Harvard Catalyst Profiles

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Marian DiFiglia, Ph.D.

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Research
The research activities and funding listed below are automatically derived from NIH ExPORTER and other sources, which might result in incorrect or missing items. Faculty can login to make corrections and additions.
  1. R01NS074381 (DIFIGLIA, MARIAN) Sep 30, 2011 - Jun 30, 2015
    NIH
    Increase Rab11 Activity as HD Therapy
    Role: Principal Investigator
  2. S10RR023594 (DIFIGLIA, MARIAN) Feb 1, 2010 - Jan 31, 2012
    NIH
    Request for electron microscope
    Role: Principal Investigator
  3. R01NS035711 (DIFIGLIA, MARIAN) Aug 15, 1998 - Jul 31, 2007
    NIH
    Role of Huntingtin in Vesicle Transport
    Role: Principal Investigator
  4. P01NS031579 (DIFIGLIA, MARIAN) Jan 1, 1994 - Dec 31, 1999
    NIH/NINDS
    CORE--RECEPTOR ANTIBODIES AND OLIGONUCLEOTIDE PROBES PREPARATION
    Role: Principal Investigator
  5. P50NS016367 (GUSELLA, JAMES F) Jul 1, 1980 - Mar 31, 2012
    NIH
    Huntington's Disease Center Without Walls
    Role: Co-Principal Investigator

Bibliographic
Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
  1. Oikemus SR, Pfister EL, Sapp E, Chase KO, Kennington LA, Hudgens E, Miller R, Zhu LJ, Chaudhary A, Mick EO, Sena-Esteves M, Wolfe SA, DiFiglia M, Aronin N, Brodsky MH. Allele-Specific Knockdown of Mutant Huntingtin Protein via Editing at Coding Region Single Nucleotide Polymorphism Heterozygosities. Hum Gene Ther. 2022 01; 33(1-2):25-36. PMID: 34376056.
    Citations: 2     Fields:    Translation:Animals
  2. Yamada K, Hildebrand S, Davis SM, Miller R, Conroy F, Sapp E, Caiazzi J, Alterman JF, Roux L, Echeverria D, Hassler MR, Pfister EL, DiFiglia M, Aronin N, Khvorova A. Structurally constrained phosphonate internucleotide linkage impacts oligonucleotide-enzyme interaction, and modulates siRNA activity and allele specificity. Nucleic Acids Res. 2021 12 02; 49(21):12069-12088. PMID: 34850120.
    Citations:    Fields:    Translation:Humans
  3. Petrozziello T, Bordt EA, Mills AN, Kim SE, Sapp E, Devlin BA, Obeng-Marnu AA, Farhan SMK, Amaral AC, Dujardin S, Dooley PM, Henstridge C, Oakley DH, Neueder A, Hyman BT, Spires-Jones TL, Bilbo SD, Vakili K, Cudkowicz ME, Berry JD, DiFiglia M, Silva MC, Haggarty SJ, Sadri-Vakili G. Targeting Tau Mitigates Mitochondrial Fragmentation and Oxidative Stress in Amyotrophic Lateral Sclerosis. Mol Neurobiol. 2022 Jan; 59(1):683-702. PMID: 34757590.
    Citations: 2     Fields:    Translation:HumansCells
  4. Iuliano M, Seeley C, Sapp E, Jones EL, Martin C, Li X, DiFiglia M, Kegel-Gleason KB. Disposition of Proteins and Lipids in Synaptic Membrane Compartments Is Altered in Q175/Q7 Huntington's Disease Mouse Striatum. Front Synaptic Neurosci. 2021; 13:618391. PMID: 33815086.
    Citations: 2     
  5. Fernandez-Cerado C, Legarda GP, Velasco-Andrada MS, Aguil A, Ganza-Bautista NG, Lagarde JBB, Soria J, Jamora RDG, Acuña PJ, Vanderburg C, Sapp E, DiFiglia M, Murcar MG, Campion L, Ozelius LJ, Alessi AK, Singh-Bains MK, Waldvogel HJ, Faull RLM, Macalintal-Canlas R, Muñoz EL, Penney EB, Ang MA, Diesta CCE, Bragg DC, Acuña-Sunshine G. Promise and challenges of dystonia brain banking: establishing a human tissue repository for studies of X-Linked Dystonia-Parkinsonism. J Neural Transm (Vienna). 2021 04; 128(4):575-587. PMID: 33439365.
    Citations: 3     Fields:    Translation:Humans
  6. Ke Y, Weng M, Chhetri G, Usman M, Li Y, Yu Q, Ding Y, Wang Z, Wang X, Sultana P, DiFiglia M, Li X. Trappc9 deficiency in mice impairs learning and memory by causing imbalance of dopamine D1 and D2 neurons. Sci Adv. 2020 11; 6(47). PMID: 33208359.
    Citations: 4     Fields:    
  7. DiFiglia M. An early start to Huntington's disease. Science. 2020 08 14; 369(6505):771-772. PMID: 32792383.
    Citations: 4     Fields:    Translation:Humans
  8. Sapp E, Seeley C, Iuliano M, Weisman E, Vodicka P, DiFiglia M, Kegel-Gleason KB. Protein changes in synaptosomes of Huntington's disease knock-in mice are dependent on age and brain region. Neurobiol Dis. 2020 07; 141:104950. PMID: 32439598.
    Citations: 8     Fields:    Translation:AnimalsCells
  9. Wang X, Weng M, Ke Y, Sapp E, DiFiglia M, Li X. Kalirin Interacts with TRAPP and Regulates Rab11 and Endosomal Recycling. Cells. 2020 05 04; 9(5). PMID: 32375403.
    Citations: 1     Fields:    Translation:HumansAnimalsCells
  10. Aviolat H, Pinto RM, Godschall E, Murtha R, Richey HE, Sapp E, Vodicka P, Wheeler VC, Kegel-Gleason KB, DiFiglia M. Assessing average somatic CAG repeat instability at the protein level. Sci Rep. 2019 12 16; 9(1):19152. PMID: 31844074.
    Citations: 5     Fields:    Translation:HumansAnimalsCells
  11. Li Z, Wang C, Wang Z, Zhu C, Li J, Sha T, Ma L, Gao C, Yang Y, Sun Y, Wang J, Sun X, Lu C, Difiglia M, Mei Y, Ding C, Luo S, Dang Y, Ding Y, Fei Y, Lu B. Allele-selective lowering of mutant HTT protein by HTT-LC3 linker compounds. Nature. 2019 11; 575(7781):203-209. PMID: 31666698.
    Citations: 65     Fields:    Translation:HumansAnimalsCells
  12. Alterman JF, Godinho BMDC, Hassler MR, Ferguson CM, Echeverria D, Sapp E, Haraszti RA, Coles AH, Conroy F, Miller R, Roux L, Yan P, Knox EG, Turanov AA, King RM, Gernoux G, Mueller C, Gray-Edwards HL, Moser RP, Bishop NC, Jaber SM, Gounis MJ, Sena-Esteves M, Pai AA, DiFiglia M, Aronin N, Khvorova A. A divalent siRNA chemical scaffold for potent and sustained modulation of gene expression throughout the central nervous system. Nat Biotechnol. 2019 08; 37(8):884-894. PMID: 31375812.
    Citations: 36     Fields:    Translation:Animals
  13. Haraszti RA, Miller R, Dubuke ML, Rockwell HE, Coles AH, Sapp E, Didiot MC, Echeverria D, Stoppato M, Sere YY, Leszyk J, Alterman JF, Godinho BMDC, Hassler MR, McDaniel J, Narain NR, Wollacott R, Wang Y, Shaffer SA, Kiebish MA, DiFiglia M, Aronin N, Khvorova A. Serum Deprivation of Mesenchymal Stem Cells Improves Exosome Activity and Alters Lipid and Protein Composition. iScience. 2019 Jun 28; 16:230-241. PMID: 31195240.
    Citations: 18     
  14. Tousley A, Iuliano M, Weisman E, Sapp E, Richardson H, Vodicka P, Alexander J, Aronin N, DiFiglia M, Kegel-Gleason KB. Huntingtin associates with the actin cytoskeleton and a-actinin isoforms to influence stimulus dependent morphology changes. PLoS One. 2019; 14(2):e0212337. PMID: 30768638.
    Citations: 11     Fields:    Translation:HumansCells
  15. Tousley A, Iuliano M, Weisman E, Sapp E, Zhang N, Vodicka P, Alexander J, Aviolat H, Gatune L, Reeves P, Li X, Khvorova A, Ellerby LM, Aronin N, DiFiglia M, Kegel-Gleason KB. Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington's Disease. J Huntingtons Dis. 2019; 8(1):53-69. PMID: 30594931.
    Citations: 7     Fields:    Translation:HumansAnimalsCells
  16. Haraszti RA, Miller R, Stoppato M, Sere YY, Coles A, Didiot MC, Wollacott R, Sapp E, Dubuke ML, Li X, Shaffer SA, DiFiglia M, Wang Y, Aronin N, Khvorova A. Exosomes Produced from 3D Cultures of MSCs by Tangential Flow Filtration Show Higher Yield and Improved Activity. Mol Ther. 2018 12 05; 26(12):2838-2847. PMID: 30341012.
    Citations: 78     Fields:    Translation:AnimalsCells
  17. Didiot MC, Ferguson CM, Ly S, Coles AH, Smith AO, Bicknell AA, Hall LM, Sapp E, Echeverria D, Pai AA, DiFiglia M, Moore MJ, Hayward LJ, Aronin N, Khvorova A. Nuclear Localization of Huntingtin mRNA Is Specific to Cells of Neuronal Origin. Cell Rep. 2018 09 04; 24(10):2553-2560.e5. PMID: 30184490.
    Citations: 9     Fields:    Translation:HumansAnimalsCells
  18. Godinho BMDC, Henninger N, Bouley J, Alterman JF, Haraszti RA, Gilbert JW, Sapp E, Coles AH, Biscans A, Nikan M, Echeverria D, DiFiglia M, Aronin N, Khvorova A. Transvascular Delivery of Hydrophobically Modified siRNAs: Gene Silencing in the Rat Brain upon Disruption of the Blood-Brain Barrier. Mol Ther. 2018 11 07; 26(11):2580-2591. PMID: 30143435.
    Citations: 15     Fields:    Translation:HumansAnimalsCells
  19. Mueller KA, Glajch KE, Huizenga MN, Wilson RA, Granucci EJ, Dios AM, Tousley AR, Iuliano M, Weisman E, LaQuaglia MJ, DiFiglia M, Kegel-Gleason K, Vakili K, Sadri-Vakili G. Hippo Signaling Pathway Dysregulation in Human Huntington's Disease Brain and Neuronal Stem Cells. Sci Rep. 2018 07 27; 8(1):11355. PMID: 30054496.
    Citations: 22     Fields:    Translation:HumansAnimalsCells
  20. Haraszti RA, Miller R, Didiot MC, Biscans A, Alterman JF, Hassler MR, Roux L, Echeverria D, Sapp E, DiFiglia M, Aronin N, Khvorova A. Optimized Cholesterol-siRNA Chemistry Improves Productive Loading onto Extracellular Vesicles. Mol Ther. 2018 08 01; 26(8):1973-1982. PMID: 29937418.
    Citations: 23     Fields:    Translation:HumansAnimalsCells
  21. McClory H, Wang X, Sapp E, Gatune LW, Iuliano M, Wu CY, Nathwani G, Kegel-Gleason KB, DiFiglia M, Li X. The COOH-terminal domain of huntingtin interacts with RhoGEF kalirin and modulates cell survival. Sci Rep. 2018 05 22; 8(1):8000. PMID: 29789657.
    Citations: 5     Fields:    Translation:HumansAnimalsCells
  22. Pfister EL, DiNardo N, Mondo E, Borel F, Conroy F, Fraser C, Gernoux G, Han X, Hu D, Johnson E, Kennington L, Liu P, Reid SJ, Sapp E, Vodicka P, Kuchel T, Morton AJ, Howland D, Moser R, Sena-Esteves M, Gao G, Mueller C, DiFiglia M, Aronin N. Artificial miRNAs Reduce Human Mutant Huntingtin Throughout the Striatum in a Transgenic Sheep Model of Huntington's Disease. Hum Gene Ther. 2018 06; 29(6):663-673. PMID: 29207890.
    Citations: 35     Fields:    Translation:HumansAnimalsCells
  23. Kovalenko M, Milnerwood A, Giordano J, St Claire J, Guide JR, Stromberg M, Gillis T, Sapp E, DiFiglia M, MacDonald ME, Carroll JB, Lee JM, Tappan S, Raymond L, Wheeler VC. HttQ111/+ Huntington's Disease Knock-in Mice Exhibit Brain Region-Specific Morphological Changes and Synaptic Dysfunction. J Huntingtons Dis. 2018; 7(1):17-33. PMID: 29480209.
    Citations: 8     Fields:    Translation:AnimalsCells
  24. Mondo E, Moser R, Gao G, Mueller C, Sena-Esteves M, Sapp E, Pfister E, O'Connell D, Takle K, Erger KE, Liu W, Conlon TJ, DiFiglia M, Gounis MJ, Aronin N. Selective Neuronal Uptake and Distribution of AAVrh8, AAV9, and AAVrh10 in Sheep After Intra-Striatal Administration. J Huntingtons Dis. 2018; 7(4):309-319. PMID: 30320596.
    Citations: 3     Fields:    Translation:HumansAnimalsCells
  25. Valencia A, Sapp E, Kimm JS, McClory H, Reeves PB, Alexander J, Ansong KA, Masso N, Frosch MP, Kegel KB, Li X, DiFiglia M. Retracted: Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease. Hum Mol Genet. 2017 11 01; 26(21):4314. PMID: 28973680.
    Citations:    Fields:    
  26. Fu Y, Wu P, Pan Y, Sun X, Yang H, Difiglia M, Lu B. A toxic mutant huntingtin species is resistant to selective autophagy. Nat Chem Biol. 2017 Nov; 13(11):1152-1154. PMID: 28869595.
    Citations: 16     Fields:    Translation:HumansCells
  27. Quinti L, Dayalan Naidu S, Träger U, Chen X, Kegel-Gleason K, Llères D, Connolly C, Chopra V, Low C, Moniot S, Sapp E, Tousley AR, Vodicka P, Van Kanegan MJ, Kaltenbach LS, Crawford LA, Fuszard M, Higgins M, Miller JRC, Farmer RE, Potluri V, Samajdar S, Meisel L, Zhang N, Snyder A, Stein R, Hersch SM, Ellerby LM, Weerapana E, Schwarzschild MA, Steegborn C, Leavitt BR, Degterev A, Tabrizi SJ, Lo DC, DiFiglia M, Thompson LM, Dinkova-Kostova AT, Kazantsev AG. KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients. Proc Natl Acad Sci U S A. 2017 06 06; 114(23):E4676-E4685. PMID: 28533375.
    Citations: 34     Fields:    Translation:HumansAnimalsCells
  28. Nikan M, Osborn MF, Coles AH, Biscans A, Godinho BMDC, Haraszti RA, Sapp E, Echeverria D, DiFiglia M, Aronin N, Khvorova A. Synthesis and Evaluation of Parenchymal Retention and Efficacy of a Metabolically Stable O-Phosphocholine-N-docosahexaenoyl-l-serine siRNA Conjugate in Mouse Brain. Bioconjug Chem. 2017 06 21; 28(6):1758-1766. PMID: 28462988.
    Citations: 20     Fields:    Translation:Animals
  29. Haraszti RA, Didiot MC, Sapp E, Leszyk J, Shaffer SA, Rockwell HE, Gao F, Narain NR, DiFiglia M, Kiebish MA, Aronin N, Khvorova A. High-resolution proteomic and lipidomic analysis of exosomes and microvesicles from different cell sources. J Extracell Vesicles. 2016; 5:32570. PMID: 27863537.
    Citations: 208     Fields:    
  30. Vodicka P, Chase K, Iuliano M, Tousley A, Valentine DT, Sapp E, Kegel-Gleason KB, Sena-Esteves M, Aronin N, DiFiglia M. Autophagy Activation by Transcription Factor EB (TFEB) in Striatum of HDQ175/Q7 Mice. J Huntingtons Dis. 2016 10 01; 5(3):249-260. PMID: 27689619.
    Citations: 14     Fields:    Translation:HumansAnimals
  31. Keeler AM, Sapp E, Chase K, Sottosanti E, Danielson E, Pfister E, Stoica L, DiFiglia M, Aronin N, Sena-Esteves M. Cellular Analysis of Silencing the Huntington's Disease Gene Using AAV9 Mediated Delivery of Artificial Micro RNA into the Striatum of Q140/Q140 Mice. J Huntingtons Dis. 2016 10 01; 5(3):239-248. PMID: 27689620.
    Citations: 15     Fields:    Translation:HumansAnimalsCells
  32. Didiot MC, Hall LM, Coles AH, Haraszti RA, Godinho BM, Chase K, Sapp E, Ly S, Alterman JF, Hassler MR, Echeverria D, Raj L, Morrissey DV, DiFiglia M, Aronin N, Khvorova A. Exosome-mediated Delivery of Hydrophobically Modified siRNA for Huntingtin mRNA Silencing. Mol Ther. 2016 10; 24(10):1836-1847. PMID: 27506293.
    Citations: 125     Fields:    Translation:HumansAnimalsCells
  33. Vodicka P, Chase K, Iuliano M, Valentine DT, Sapp E, Lu B, Kegel-Gleason KB, Sena-Esteves M, Aronin N, DiFiglia M. Effects of Exogenous NUB1 Expression in the Striatum of HDQ175/Q7 Mice. J Huntingtons Dis. 2016 06 13; 5(2):163-74. PMID: 27314618.
    Citations: 3     Fields:    Translation:HumansAnimalsCells
  34. Macakova M, Bohuslavova B, Vochozkova P, Pavlok A, Sedlackova M, Vidinska D, Vochyanova K, Liskova I, Valekova I, Baxa M, Ellederova Z, Klima J, Juhas S, Juhasova J, Klouckova J, Haluzik M, Klempir J, Hansikova H, Spacilova J, Collins R, Blumenthal I, Talkowski M, Gusella JF, Howland DS, DiFiglia M, Motlik J. Mutated Huntingtin Causes Testicular Pathology in Transgenic Minipig Boars. Neurodegener Dis. 2016; 16(3-4):245-59. PMID: 26959244.
    Citations: 8     Fields:    Translation:HumansAnimalsCells
  35. Liu W, Pfister EL, Kennington LA, Chase KO, Mueller C, DiFiglia M, Aronin N. Does the Mutant CAG Expansion in Huntingtin mRNA Interfere with Exonucleolytic Cleavage of its First Exon? J Huntingtons Dis. 2016; 5(1):33-8. PMID: 27003665.
    Citations: 2     Fields:    Translation:AnimalsCells
  36. Choudhury SR, Harris AF, Cabral DJ, Keeler AM, Sapp E, Ferreira JS, Gray-Edwards HL, Johnson JA, Johnson AK, Su Q, Stoica L, DiFiglia M, Aronin N, Martin DR, Gao G, Sena-Esteves M. Widespread Central Nervous System Gene Transfer and Silencing After Systemic Delivery of Novel AAV-AS Vector. Mol Ther. 2016 Apr; 24(4):726-35. PMID: 26708003.
    Citations: 46     Fields:    Translation:AnimalsCells
  37. Alterman JF, Hall LM, Coles AH, Hassler MR, Didiot MC, Chase K, Abraham J, Sottosanti E, Johnson E, Sapp E, Osborn MF, Difiglia M, Aronin N, Khvorova A. Hydrophobically Modified siRNAs Silence Huntingtin mRNA in Primary Neurons and Mouse Brain. Mol Ther Nucleic Acids. 2015 Dec 01; 4:e266. PMID: 26623938.
    Citations: 51     
  38. Yao Y, Cui X, Al-Ramahi I, Sun X, Li B, Hou J, Difiglia M, Palacino J, Wu ZY, Ma L, Botas J, Lu B. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. Elife. 2015 Mar 04; 4. PMID: 25738228.
    Citations: 33     Fields:    Translation:HumansAnimalsCells
  39. Vodicka P, Mo S, Tousley A, Green KM, Sapp E, Iuliano M, Sadri-Vakili G, Shaffer SA, Aronin N, DiFiglia M, Kegel-Gleason KB. Mass Spectrometry Analysis of Wild-Type and Knock-in Q140/Q140 Huntington's Disease Mouse Brains Reveals Changes in Glycerophospholipids Including Alterations in Phosphatidic Acid and Lyso-Phosphatidic Acid. J Huntingtons Dis. 2015; 4(2):187-201. PMID: 26397899.
    Citations: 6     Fields:    Translation:AnimalsCells
  40. Johnson E, Chase K, McGowan S, Mondo E, Pfister E, Mick E, Friedline RH, Kim JK, Sapp E, DiFiglia M, Aronin N. Safety of Striatal Infusion of siRNA in a Transgenic Huntington's Disease Mouse Model. J Huntingtons Dis. 2015; 4(3):219-229. PMID: 26444021.
    Citations: 2     Fields:    Translation:Animals
  41. McClory H, Williams D, Sapp E, Gatune LW, Wang P, DiFiglia M, Li X. Glucose transporter 3 is a rab11-dependent trafficking cargo and its transport to the cell surface is reduced in neurons of CAG140 Huntington's disease mice. Acta Neuropathol Commun. 2014 Dec 20; 2:179. PMID: 25526803.
    Citations: 17     Fields:    Translation:HumansAnimalsCells
  42. Grishchuk Y, Sri S, Rudinskiy N, Ma W, Stember KG, Cottle MW, Sapp E, Difiglia M, Muzikansky A, Betensky RA, Wong AM, Bacskai BJ, Hyman BT, Kelleher RJ, Cooper JD, Slaugenhaupt SA. Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV. Acta Neuropathol Commun. 2014 Sep 09; 2:133. PMID: 25200117.
    Citations: 20     Fields:    Translation:AnimalsCells
  43. Aronin N, DiFiglia M. Huntingtin-lowering strategies in Huntington's disease: antisense oligonucleotides, small RNAs, and gene editing. Mov Disord. 2014 Sep 15; 29(11):1455-61. PMID: 25164989.
    Citations: 39     Fields:    Translation:HumansAnimalsCells
  44. Sheng R, Zhang TT, Felice VD, Qin T, Qin ZH, Smith CD, Sapp E, Difiglia M, Waeber C. Preconditioning stimuli induce autophagy via sphingosine kinase 2 in mouse cortical neurons. J Biol Chem. 2014 Jul 25; 289(30):20845-57. PMID: 24928515.
    Citations: 20     Fields:    Translation:AnimalsCells
  45. Yu S, Liang Y, Palacino J, Difiglia M, Lu B. Drugging unconventional targets: insights from Huntington's disease. Trends Pharmacol Sci. 2014 Feb; 35(2):53-62. PMID: 24388390.
    Citations: 10     Fields:    Translation:HumansAnimals
  46. Vodicka P, Lim J, Williams DT, Kegel KB, Chase K, Park H, Marchionini D, Wilkinson S, Mead T, Birch H, Yates D, Lyons K, Dominguez C, Beconi M, Yue Z, Aronin N, DiFiglia M. Assessment of chloroquine treatment for modulating autophagy flux in brain of WT and HD mice. J Huntingtons Dis. 2014; 3(2):159-74. PMID: 25062859.
    Citations: 8     Fields:    Translation:Animals
  47. O'Rourke JG, Gareau JR, Ochaba J, Song W, Raskó T, Reverter D, Lee J, Monteys AM, Pallos J, Mee L, Vashishtha M, Apostol BL, Nicholson TP, Illes K, Zhu YZ, Dasso M, Bates GP, Difiglia M, Davidson B, Wanker EE, Marsh JL, Lima CD, Steffan JS, Thompson LM. SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Rep. 2013 Jul 25; 4(2):362-75. PMID: 23871671.
    Citations: 48     Fields:    Translation:HumansAnimalsCells
  48. Lu B, Al-Ramahi I, Valencia A, Wang Q, Berenshteyn F, Yang H, Gallego-Flores T, Ichcho S, Lacoste A, Hild M, Difiglia M, Botas J, Palacino J. Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nat Neurosci. 2013 May; 16(5):562-70. PMID: 23525043.
    Citations: 47     Fields:    Translation:HumansAnimalsCells
  49. Valencia A, Sapp E, Kimm JS, McClory H, Ansong KA, Yohrling G, Kwak S, Kegel KB, Green KM, Shaffer SA, Aronin N, DiFiglia M. Striatal synaptosomes from Hdh140Q/140Q knock-in mice have altered protein levels, novel sites of methionine oxidation, and excess glutamate release after stimulation. J Huntingtons Dis. 2013; 2(4):459-75. PMID: 24696705.
    Citations: 17     Fields:    Translation:AnimalsCells
  50. Liu W, Chaurette J, Pfister EL, Kennington LA, Chase KO, Bullock J, Vonsattel JP, Faull RL, Macdonald D, DiFiglia M, Zamore PD, Aronin N. Increased Steady-State Mutant Huntingtin mRNA in Huntington's Disease Brain. J Huntingtons Dis. 2013; 2(4):491-500. PMID: 25062733.
    Citations: 8     Fields:    Translation:HumansCells
  51. Baxa M, Hruska-Plochan M, Juhas S, Vodicka P, Pavlok A, Juhasova J, Miyanohara A, Nejime T, Klima J, Macakova M, Marsala S, Weiss A, Kubickova S, Musilova P, Vrtel R, Sontag EM, Thompson LM, Schier J, Hansikova H, Howland DS, Cattaneo E, DiFiglia M, Marsala M, Motlik J. A transgenic minipig model of Huntington's Disease. J Huntingtons Dis. 2013; 2(1):47-68. PMID: 25063429.
    Citations: 36     Fields:    Translation:AnimalsCells
  52. Valencia A, Sapp E, Kimm JS, McClory H, Reeves PB, Alexander J, Ansong KA, Masso N, Frosch MP, Kegel KB, Li X, DiFiglia M. Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease. Hum Mol Genet. 2013 Mar 15; 22(6):1112-31. PMID: 23223017.
    Citations: 55     Fields:    Translation:HumansAnimalsCells
  53. Qi L, Zhang XD, Wu JC, Lin F, Wang J, DiFiglia M, Qin ZH. The role of chaperone-mediated autophagy in huntingtin degradation. PLoS One. 2012; 7(10):e46834. PMID: 23071649.
    Citations: 62     Fields:    Translation:HumansAnimalsCells
  54. Wu JC, Qi L, Wang Y, Kegel KB, Yoder J, Difiglia M, Qin ZH, Lin F. The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1. Acta Pharmacol Sin. 2012 Jun; 33(6):743-51. PMID: 22543707.
    Citations: 19     Fields:    Translation:HumansAnimalsCells
  55. Li X, Valencia A, McClory H, Sapp E, Kegel KB, Difiglia M. Deficient Rab11 activity underlies glucose hypometabolism in primary neurons of Huntington's disease mice. Biochem Biophys Res Commun. 2012 May 18; 421(4):727-30. PMID: 22542623.
    Citations: 14     Fields:    Translation:AnimalsCells
  56. Ritch JJ, Valencia A, Alexander J, Sapp E, Gatune L, Sangrey GR, Sinha S, Scherber CM, Zeitlin S, Sadri-Vakili G, Irimia D, Difiglia M, Kegel KB. Multiple phenotypes in Huntington disease mouse neural stem cells. Mol Cell Neurosci. 2012 May; 50(1):70-81. PMID: 22508027.
    Citations: 28     Fields:    Translation:AnimalsCells
  57. Sapp E, Valencia A, Li X, Aronin N, Kegel KB, Vonsattel JP, Young AB, Wexler N, DiFiglia M. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer. J Biol Chem. 2012 Apr 13; 287(16):13487-99. PMID: 22375012.
    Citations: 12     Fields:    Translation:HumansAnimalsCells
  58. Wang L, Lin F, Wang J, Wu J, Han R, Zhu L, Difiglia M, Qin Z. Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF. Brain Res. 2012 Apr 17; 1449:69-82. PMID: 22410294.
    Citations: 16     Fields:    Translation:AnimalsCells
  59. Chen LL, Wu JC, Wang LH, Wang J, Qin ZH, Difiglia M, Lin F. Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes. Acta Pharmacol Sin. 2012 Mar; 33(3):385-92. PMID: 22266730.
    Citations: 6     Fields:    Translation:AnimalsCells
  60. Wang L, Lin F, Wang J, Wu J, Han R, Zhu L, Zhang G, DiFiglia M, Qin Z. Truncated N-terminal huntingtin fragment with expanded-polyglutamine (htt552-100Q) suppresses brain-derived neurotrophic factor transcription in astrocytes. Acta Biochim Biophys Sin (Shanghai). 2012 Mar; 44(3):249-58. PMID: 22234237.
    Citations: 11     Fields:    Translation:HumansAnimalsCells
  61. Valencia A, Sapp E, Reeves PB, Alexander J, Masso N, Li X, Kegel KB, DiFiglia M. Reagents that block neuronal death from Huntington's disease also curb oxidative stress. Neuroreport. 2012 Jan 04; 23(1):10-5. PMID: 22045254.
    Citations: 10     Fields:    Translation:AnimalsCells
  62. Li X, DiFiglia M. The recycling endosome and its role in neurological disorders. Prog Neurobiol. 2012 May; 97(2):127-41. PMID: 22037413.
    Citations: 29     Fields:    Translation:HumansAnimalsCells
  63. Fox JH, Connor T, Stiles M, Kama J, Lu Z, Dorsey K, Lieberman G, Liebermann G, Sapp E, Cherny RA, Banks M, Volitakis I, DiFiglia M, Berezovska O, Bush AI, Hersch SM. Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein. J Biol Chem. 2011 May 20; 286(20):18320-30. PMID: 21454633.
    Citations: 23     Fields:    Translation:HumansAnimalsCells
  64. Moscovitch-Lopatin M, Weiss A, Rosas HD, Ritch J, Doros G, Kegel KB, Difiglia M, Kuhn R, Bilbe G, Paganetti P, Hersch S. Optimization of an HTRF Assay for the Detection of Soluble Mutant Huntingtin in Human Buffy Coats: A Potential Biomarker in Blood for Huntington Disease. PLoS Curr. 2010 Dec 29; 2:RRN1205. PMID: 21278900.
    Citations: 10     
  65. Kegel KB, Sapp E, Alexander J, Reeves P, Bleckmann D, Sobin L, Masso N, Valencia A, Jeong H, Krainc D, Palacino J, Curtis D, Kuhn R, Betschart C, Sena-Esteves M, Aronin N, Paganetti P, Difiglia M. Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors. Mol Neurodegener. 2010 Dec 14; 5:58. PMID: 21156064.
    Citations: 11     Fields:    
  66. Fox JH, Connor T, Chopra V, Dorsey K, Kama JA, Bleckmann D, Betschart C, Hoyer D, Frentzel S, Difiglia M, Paganetti P, Hersch SM. The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease. Mol Neurodegener. 2010 Jun 22; 5:26. PMID: 20569486.
    Citations: 47     Fields:    
  67. Li X, Valencia A, Sapp E, Masso N, Alexander J, Reeves P, Kegel KB, Aronin N, Difiglia M. Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease. J Neurosci. 2010 Mar 31; 30(13):4552-61. PMID: 20357106.
    Citations: 81     Fields:    Translation:HumansAnimalsCells
  68. Legleiter J, Mitchell E, Lotz GP, Sapp E, Ng C, DiFiglia M, Thompson LM, Muchowski PJ. Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. J Biol Chem. 2010 May 07; 285(19):14777-90. PMID: 20220138.
    Citations: 89     Fields:    Translation:HumansAnimalsCells
  69. Valencia A, Reeves PB, Sapp E, Li X, Alexander J, Kegel KB, Chase K, Aronin N, DiFiglia M. Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease. J Neurosci Res. 2010 Jan; 88(1):179-90. PMID: 19642201.
    Citations: 39     Fields:    Translation:AnimalsCells
  70. Li X, Standley C, Sapp E, Valencia A, Qin ZH, Kegel KB, Yoder J, Comer-Tierney LA, Esteves M, Chase K, Alexander J, Masso N, Sobin L, Bellve K, Tuft R, Lifshitz L, Fogarty K, Aronin N, DiFiglia M. Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity. Mol Cell Biol. 2009 Nov; 29(22):6106-16. PMID: 19752198.
    Citations: 51     Fields:    Translation:HumansCells
  71. Li X, Sapp E, Chase K, Comer-Tierney LA, Masso N, Alexander J, Reeves P, Kegel KB, Valencia A, Esteves M, Aronin N, Difiglia M. Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease. Neurobiol Dis. 2009 Nov; 36(2):374-83. PMID: 19699304.
    Citations: 34     Fields:    Translation:AnimalsCells
  72. Weiss A, Abramowski D, Bibel M, Bodner R, Chopra V, DiFiglia M, Fox J, Kegel K, Klein C, Grueninger S, Hersch S, Housman D, Régulier E, Rosas HD, Stefani M, Zeitlin S, Bilbe G, Paganetti P. Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Anal Biochem. 2009 Dec 01; 395(1):8-15. PMID: 19664996.
    Citations: 58     Fields:    Translation:HumansAnimalsCells
  73. Kegel KB, Schewkunow V, Sapp E, Masso N, Wanker EE, DiFiglia M, Goldmann WH. Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers. Biochem Biophys Res Commun. 2009 Sep 25; 387(3):472-5. PMID: 19607813.
    Citations: 10     Fields:    Translation:HumansCells
  74. Kegel KB, Sapp E, Alexander J, Valencia A, Reeves P, Li X, Masso N, Sobin L, Aronin N, DiFiglia M. Polyglutamine expansion in huntingtin alters its interaction with phospholipids. J Neurochem. 2009 Sep; 110(5):1585-97. PMID: 19566678.
    Citations: 37     Fields:    Translation:HumansAnimalsCells
  75. Pfister EL, Kennington L, Straubhaar J, Wagh S, Liu W, DiFiglia M, Landwehrmeyer B, Vonsattel JP, Zamore PD, Aronin N. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol. 2009 May 12; 19(9):774-8. PMID: 19361997.
    Citations: 124     Fields:    Translation:Humans
  76. Li X, Sapp E, Valencia A, Kegel KB, Qin ZH, Alexander J, Masso N, Reeves P, Ritch JJ, Zeitlin S, Aronin N, Difiglia M. A function of huntingtin in guanine nucleotide exchange on Rab11. Neuroreport. 2008 Oct 29; 19(16):1643-7. PMID: 18845944.
    Citations: 31     Fields:    Translation:AnimalsCells
  77. Klionsky DJ, Abeliovich H, Agostinis P, Agrawal DK, Aliev G, Askew DS, Baba M, Baehrecke EH, Bahr BA, Ballabio A, Bamber BA, Bassham DC, Bergamini E, Bi X, Biard-Piechaczyk M, Blum JS, Bredesen DE, Brodsky JL, Brumell JH, Brunk UT, Bursch W, Camougrand N, Cebollero E, Cecconi F, Chen Y, Chin LS, Choi A, Chu CT, Chung J, Clarke PG, Clark RS, Clarke SG, Clavé C, Cleveland JL, Codogno P, Colombo MI, Coto-Montes A, Cregg JM, Cuervo AM, Debnath J, Demarchi F, Dennis PB, Dennis PA, Deretic V, Devenish RJ, Di Sano F, Dice JF, Difiglia M, Dinesh-Kumar S, Distelhorst CW, Djavaheri-Mergny M, Dorsey FC, Dröge W, Dron M, Dunn WA, Duszenko M, Eissa NT, Elazar Z, Esclatine A, Eskelinen EL, Fésüs L, Finley KD, Fuentes JM, Fueyo J, Fujisaki K, Galliot B, Gao FB, Gewirtz DA, Gibson SB, Gohla A, Goldberg AL, Gonzalez R, González-Estévez C, Gorski S, Gottlieb RA, Häussinger D, He YW, Heidenreich K, Hill JA, Høyer-Hansen M, Hu X, Huang WP, Iwasaki A, Jäättelä M, Jackson WT, Jiang X, Jin S, Johansen T, Jung JU, Kadowaki M, Kang C, Kelekar A, Kessel DH, Kiel JA, Kim HP, Kimchi A, Kinsella TJ, Kiselyov K, Kitamoto K, Knecht E, Komatsu M, Kominami E, Kondo S, Kovács AL, Kroemer G, Kuan CY, Kumar R, Kundu M, Landry J, Laporte M, Le W, Lei HY, Lenardo MJ, Levine B, Lieberman A, Lim KL, Lin FC, Liou W, Liu LF, Lopez-Berestein G, López-Otín C, Lu B, Macleod KF, Malorni W, Martinet W, Matsuoka K, Mautner J, Meijer AJ, Meléndez A, Michels P, Miotto G, Mistiaen WP, Mizushima N, Mograbi B, Monastyrska I, Moore MN, Moreira PI, Moriyasu Y, Motyl T, Münz C, Murphy LO, Naqvi NI, Neufeld TP, Nishino I, Nixon RA, Noda T, Nürnberg B, Ogawa M, Oleinick NL, Olsen LJ, Ozpolat B, Paglin S, Palmer GE, Papassideri I, Parkes M, Perlmutter DH, Perry G, Piacentini M, Pinkas-Kramarski R, Prescott M, Proikas-Cezanne T, Raben N, Rami A, Reggiori F, Rohrer B, Rubinsztein DC, Ryan KM, Sadoshima J, Sakagami H, Sakai Y, Sandri M, Sasakawa C, Sass M, Schneider C, Seglen PO, Seleverstov O, Settleman J, Shacka JJ, Shapiro IM, Sibirny A, Silva-Zacarin EC, Simon HU, Simone C, Simonsen A, Smith MA, Spanel-Borowski K, Srinivas V, Steeves M, Stenmark H, Stromhaug PE, Subauste CS, Sugimoto S, Sulzer D, Suzuki T, Swanson MS, Tabas I, Takeshita F, Talbot NJ, Tallóczy Z, Tanaka K, Tanaka K, Tanida I, Taylor GS, Taylor JP, Terman A, Tettamanti G, Thompson CB, Thumm M, Tolkovsky AM, Tooze SA, Truant R, Tumanovska LV, Uchiyama Y, Ueno T, Uzcátegui NL, van der Klei I, Vaquero EC, Vellai T, Vogel MW, Wang HG, Webster P, Wiley JW, Xi Z, Xiao G, Yahalom J, Yang JM, Yap G, Yin XM, Yoshimori T, Yu L, Yue Z, Yuzaki M, Zabirnyk O, Zheng X, Zhu X, Deter RL. Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy. 2008 Feb; 4(2):151-75. PMID: 18188003.
    Citations: 1166     Fields:    Translation:HumansAnimalsCells
  78. DiFiglia M, Sena-Esteves M, Chase K, Sapp E, Pfister E, Sass M, Yoder J, Reeves P, Pandey RK, Rajeev KG, Manoharan M, Sah DW, Zamore PD, Aronin N. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc Natl Acad Sci U S A. 2007 Oct 23; 104(43):17204-9. PMID: 17940007.
    Citations: 182     Fields:    Translation:HumansAnimalsCells
  79. Kim YJ, Sapp E, Cuiffo BG, Sobin L, Yoder J, Kegel KB, Qin ZH, Detloff P, Aronin N, DiFiglia M. Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiol Dis. 2006 May; 22(2):346-56. PMID: 16423528.
    Citations: 28     Fields:    Translation:AnimalsCells
  80. Liang ZQ, Wang XX, Wang Y, Chuang DM, DiFiglia M, Chase TN, Qin ZH. Susceptibility of striatal neurons to excitotoxic injury correlates with basal levels of Bcl-2 and the induction of P53 and c-Myc immunoreactivity. Neurobiol Dis. 2005 Nov; 20(2):562-73. PMID: 15922606.
    Citations: 13     Fields:    Translation:HumansAnimalsCells
  81. Kalanithi PS, Zheng W, Kataoka Y, DiFiglia M, Grantz H, Saper CB, Schwartz ML, Leckman JF, Vaccarino FM. Altered parvalbumin-positive neuron distribution in basal ganglia of individuals with Tourette syndrome. Proc Natl Acad Sci U S A. 2005 Sep 13; 102(37):13307-12. PMID: 16131542.
    Citations: 211     Fields:    Translation:HumansCells
  82. Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, DiFiglia M. Huntingtin associates with acidic phospholipids at the plasma membrane. J Biol Chem. 2005 Oct 28; 280(43):36464-73. PMID: 16085648.
    Citations: 55     Fields:    Translation:HumansAnimalsCells
  83. Rubinsztein DC, DiFiglia M, Heintz N, Nixon RA, Qin ZH, Ravikumar B, Stefanis L, Tolkovsky A. Autophagy and its possible roles in nervous system diseases, damage and repair. Autophagy. 2005 Apr; 1(1):11-22. PMID: 16874045.
    Citations: 194     Fields:    Translation:HumansAnimalsCells
  84. Ariano MA, Cepeda C, Calvert CR, Flores-Hernández J, Hernández-Echeagaray E, Klapstein GJ, Chandler SH, Aronin N, DiFiglia M, Levine MS. Striatal potassium channel dysfunction in Huntington's disease transgenic mice. J Neurophysiol. 2005 May; 93(5):2565-74. PMID: 15625098.
    Citations: 56     Fields:    Translation:AnimalsCells
  85. Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, Kegel KB, Aronin N, DiFiglia M. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. J Neurosci. 2004 Jan 07; 24(1):269-81. PMID: 14715959.
    Citations: 61     Fields:    Translation:HumansAnimalsCells
  86. Qin ZH, Wang Y, Kegel KB, Kazantsev A, Apostol BL, Thompson LM, Yoder J, Aronin N, DiFiglia M. Autophagy regulates the processing of amino terminal huntingtin fragments. Hum Mol Genet. 2003 Dec 15; 12(24):3231-44. PMID: 14570716.
    Citations: 91     Fields:    Translation:HumansAnimalsCells
  87. Kim M, Roh JK, Yoon BW, Kang L, Kim YJ, Aronin N, DiFiglia M. Huntingtin is degraded to small fragments by calpain after ischemic injury. Exp Neurol. 2003 Sep; 183(1):109-15. PMID: 12957494.
    Citations: 12     Fields:    Translation:AnimalsCells
  88. Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM. Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. Hum Mol Genet. 2002 Aug 15; 11(17):1939-51. PMID: 12165556.
    Citations: 39     Fields:    Translation:HumansAnimals
  89. DiFiglia M. Huntingtin fragments that aggregate go their separate ways. Mol Cell. 2002 Aug; 10(2):224-5. PMID: 12191468.
    Citations: 4     Fields:    Translation:HumansAnimalsCells
  90. Ariano MA, Aronin N, Difiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, Levine MS. Striatal neurochemical changes in transgenic models of Huntington's disease. J Neurosci Res. 2002 Jun 15; 68(6):716-29. PMID: 12111832.
    Citations: 34     Fields:    Translation:AnimalsCells
  91. Petersén A, Chase K, Puschban Z, DiFiglia M, Brundin P, Aronin N. Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease. Exp Neurol. 2002 May; 175(1):297-300. PMID: 12009780.
    Citations: 17     Fields:    Translation:HumansAnimalsCells
  92. Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 01; 277(9):7466-76. PMID: 11739372.
    Citations: 88     Fields:    Translation:HumansAnimalsCells
  93. Laforet GA, Sapp E, Chase K, McIntyre C, Boyce FM, Campbell M, Cadigan BA, Warzecki L, Tagle DA, Reddy PH, Cepeda C, Calvert CR, Jokel ES, Klapstein GJ, Ariano MA, Levine MS, DiFiglia M, Aronin N. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci. 2001 Dec 01; 21(23):9112-23. PMID: 11717344.
    Citations: 93     Fields:    Translation:AnimalsCells
  94. Petersén A, Hansson O, Puschban Z, Sapp E, Romero N, Castilho RF, Sulzer D, Rice M, DiFiglia M, Przedborski S, Brundin P. Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine. Eur J Neurosci. 2001 Nov; 14(9):1425-35. PMID: 11722604.
    Citations: 17     Fields:    Translation:AnimalsCells
  95. Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, DiFiglia M. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci U S A. 2001 Oct 23; 98(22):12784-9. PMID: 11675509.
    Citations: 120     Fields:    Translation:HumansAnimalsCells
  96. Sapp E, Kegel KB, Aronin N, Hashikawa T, Uchiyama Y, Tohyama K, Bhide PG, Vonsattel JP, DiFiglia M. Early and progressive accumulation of reactive microglia in the Huntington disease brain. J Neuropathol Exp Neurol. 2001 Feb; 60(2):161-72. PMID: 11273004.
    Citations: 180     Fields:    Translation:HumansCells
  97. Qin ZH, Wang Y, Kikly KK, Sapp E, Kegel KB, Aronin N, DiFiglia M. Pro-caspase-8 is predominantly localized in mitochondria and released into cytoplasm upon apoptotic stimulation. J Biol Chem. 2001 Mar 16; 276(11):8079-86. PMID: 11102441.
    Citations: 26     Fields:    Translation:HumansCells
  98. Kegel KB, Kim M, Sapp E, McIntyre C, Castaño JG, Aronin N, DiFiglia M. Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. J Neurosci. 2000 Oct 01; 20(19):7268-78. PMID: 11007884.
    Citations: 176     Fields:    Translation:AnimalsCells
  99. DiFiglia M. Genetics of childhood disorders: X. Huntington disease. J Am Acad Child Adolesc Psychiatry. 2000 Jan; 39(1):120-2. PMID: 10638076.
    Citations:    Fields:    Translation:HumansAnimals
  100. Aronin N, Kim M, Laforet G, DiFiglia M. Are there multiple pathways in the pathogenesis of Huntington's disease? Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29; 354(1386):995-1003. PMID: 10434298.
    Citations: 15     Fields:    Translation:HumansAnimalsCells
  101. Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, DiFiglia M. Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. J Neuropathol Exp Neurol. 1999 Feb; 58(2):165-73. PMID: 10029099.
    Citations: 47     Fields:    Translation:HumansCells
  102. Kim M, Lee HS, LaForet G, McIntyre C, Martin EJ, Chang P, Kim TW, Williams M, Reddy PH, Tagle D, Boyce FM, Won L, Heller A, Aronin N, DiFiglia M. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J Neurosci. 1999 Feb 01; 19(3):964-73. PMID: 9920660.
    Citations: 74     Fields:    Translation:HumansAnimalsCells
  103. Martin EJ, Kim M, Velier J, Sapp E, Lee HS, Laforet G, Won L, Chase K, Bhide PG, Heller A, Aronin N, Difiglia M. Analysis of Huntingtin-associated protein 1 in mouse brain and immortalized striatal neurons. J Comp Neurol. 1999 Jan 25; 403(4):421-30. PMID: 9888310.
    Citations: 22     Fields:    Translation:AnimalsCells
  104. Kim M, Velier J, Chase K, Laforet G, Kalchman MA, Hayden MR, Won L, Heller A, Aronin N, Difiglia M. Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin. Neuroscience. 1999; 89(4):1159-67. PMID: 10362304.
    Citations: 14     Fields:    Translation:AnimalsCells
  105. Velier J, Kim M, Schwarz C, Kim TW, Sapp E, Chase K, Aronin N, DiFiglia M. Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways. Exp Neurol. 1998 Jul; 152(1):34-40. PMID: 9682010.
    Citations: 100     Fields:    Translation:HumansAnimalsCells
  106. Vonsattel JP, DiFiglia M. Huntington disease. J Neuropathol Exp Neurol. 1998 May; 57(5):369-84. PMID: 9596408.
    Citations: 531     Fields:    Translation:Humans
  107. Davies SW, Beardsall K, Turmaine M, DiFiglia M, Aronin N, Bates GP. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet. 1998 Jan 10; 351(9096):131-3. PMID: 9439509.
    Citations: 27     Fields:    Translation:HumansAnimalsCells
  108. Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J, Vonsattel JP, Aronin N, DiFiglia M. Huntingtin localization in brains of normal and Huntington's disease patients. Ann Neurol. 1997 Oct; 42(4):604-12. PMID: 9382472.
    Citations: 103     Fields:    Translation:HumansCells
  109. DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science. 1997 Sep 26; 277(5334):1990-3. PMID: 9302293.
    Citations: 895     Fields:    Translation:HumansCells
  110. Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, Bates GP. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell. 1997 Aug 08; 90(3):537-48. PMID: 9267033.
    Citations: 674     Fields:    Translation:HumansAnimalsCells
  111. DiFiglia M. Clinical Genetics, II. Huntington's disease: from the gene to pathophysiology. Am J Psychiatry. 1997 Aug; 154(8):1046. PMID: 9247386.
    Citations: 5     Fields:    Translation:HumansCells
  112. Block-Galarza J, Chase KO, Sapp E, Vaughn KT, Vallee RB, DiFiglia M, Aronin N. Fast transport and retrograde movement of huntingtin and HAP 1 in axons. Neuroreport. 1997 Jul 07; 8(9-10):2247-51. PMID: 9243620.
    Citations: 40     Fields:    Translation:AnimalsCells
  113. Bhide PG, Day M, Sapp E, Schwarz C, Sheth A, Kim J, Young AB, Penney J, Golden J, Aronin N, DiFiglia M. Expression of normal and mutant huntingtin in the developing brain. J Neurosci. 1996 Sep 01; 16(17):5523-35. PMID: 8757264.
    Citations: 59     Fields:    Translation:HumansAnimals
  114. Reeves SA, Ueki K, Sinha B, Difiglia M, Louis DN. Regional expression and subcellular localization of the tyrosine-specific phosphatase SH-PTP2 in the adult human nervous system. Neuroscience. 1996 Apr; 71(4):1037-42. PMID: 8684607.
    Citations: 3     Fields:    Translation:HumansCells
  115. Kanai Y, Bhide PG, DiFiglia M, Hediger MA. Neuronal high-affinity glutamate transport in the rat central nervous system. Neuroreport. 1995 Nov 27; 6(17):2357-62. PMID: 8747153.
    Citations: 17     Fields:    Translation:AnimalsCells
  116. DiFiglia M, Sapp E, Chase K, Schwarz C, Meloni A, Young C, Martin E, Vonsattel JP, Carraway R, Reeves SA, et al. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron. 1995 May; 14(5):1075-81. PMID: 7748555.
    Citations: 214     Fields:    Translation:HumansAnimalsCells
  117. Huang Q, Zhou D, Sapp E, Aizawa H, Ge P, Bird ED, Vonsattel JP, DiFiglia M. Quinolinic acid-induced increases in calbindin D28k immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease. Neuroscience. 1995 Mar; 65(2):397-407. PMID: 7777157.
    Citations: 19     Fields:    Translation:HumansAnimalsCells
  118. Sapp E, Ge P, Aizawa H, Bird E, Penney J, Young AB, Vonsattel JP, DiFiglia M. Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis. Neuroscience. 1995 Jan; 64(2):397-404. PMID: 7535402.
    Citations: 42     Fields:    Translation:Humans
  119. Vonsattel JP, Aizawa H, Ge P, DiFiglia M, McKee AC, MacDonald M, Gusella JF, Landwehrmeyer GB, Bird ED, Richardson EP, et al. An improved approach to prepare human brains for research. J Neuropathol Exp Neurol. 1995 Jan; 54(1):42-56. PMID: 7815079.
    Citations: 48     Fields:    Translation:Humans
  120. Roberts RC, Ahn A, Swartz KJ, Beal MF, DiFiglia M. Intrastriatal injections of quinolinic acid or kainic acid: differential patterns of cell survival and the effects of data analysis on outcome. Exp Neurol. 1993 Dec; 124(2):274-82. PMID: 8287926.
    Citations: 8     Fields:    Translation:AnimalsCells
  121. Lester J, Fink S, Aronin N, DiFiglia M. Colocalization of D1 and D2 dopamine receptor mRNAs in striatal neurons. Brain Res. 1993 Sep 03; 621(1):106-10. PMID: 8221060.
    Citations: 24     Fields:    Translation:AnimalsCells
  122. Zhou D, DiFiglia M. Basic fibroblast growth factor enhances the growth of postnatal neostriatal GABAergic neurons in vitro. Exp Neurol. 1993 Aug; 122(2):171-88. PMID: 8405257.
    Citations: 5     Fields:    Translation:AnimalsCells
  123. Cole DG, Growdon JH, DiFiglia M. Levodopa induction of Fos immunoreactivity in rat brain following partial and complete lesions of the substantia nigra. Exp Neurol. 1993 Apr; 120(2):223-32. PMID: 8491280.
    Citations: 2     Fields:    Translation:AnimalsCells
  124. Huang Q, Zhou D, Chase K, Gusella JF, Aronin N, DiFiglia M. Immunohistochemical localization of the D1 dopamine receptor in rat brain reveals its axonal transport, pre- and postsynaptic localization, and prevalence in the basal ganglia, limbic system, and thalamic reticular nucleus. Proc Natl Acad Sci U S A. 1992 Dec 15; 89(24):11988-92. PMID: 1281547.
    Citations: 63     Fields:    Translation:AnimalsCells
  125. Aronin N, DiFiglia M. The subcellular localization of the G-protein Gi alpha in the basal ganglia reveals its potential role in both signal transduction and vesicle trafficking. J Neurosci. 1992 Sep; 12(9):3435-44. PMID: 1527588.
    Citations: 11     Fields:    Translation:AnimalsCells
  126. Freese A, Finklestein SP, DiFiglia M. Basic fibroblast growth factor protects striatal neurons in vitro from NMDA-receptor mediated excitotoxicity. Brain Res. 1992 Mar 20; 575(2):351-5. PMID: 1349256.
    Citations: 12     Fields:    Translation:AnimalsCells
  127. Huang Q, Vonsattel JP, Schaffer PA, Martuza RL, Breakefield XO, DiFiglia M. Introduction of a foreign gene (Escherichia coli lacZ) into rat neostriatal neurons using herpes simplex virus mutants: a light and electron microscopic study. Exp Neurol. 1992 Mar; 115(3):303-16. PMID: 1311266.
    Citations: 10     Fields:    Translation:AnimalsCells
  128. Huang Q, Zhou D, DiFiglia M. Neurobiotin, a useful neuroanatomical tracer for in vivo anterograde, retrograde and transneuronal tract-tracing and for in vitro labeling of neurons. J Neurosci Methods. 1992 Jan; 41(1):31-43. PMID: 1578900.
    Citations: 14     Fields:    Translation:AnimalsCells
  129. Aronin N, Chase K, Folsom R, Christakos S, DiFiglia M. Immunoreactive calcium-binding protein (calbindin-D28k) in interneurons and trigeminothalamic neurons of the rat nucleus caudalis localized with peroxidase and immunogold methods. Synapse. 1991 Feb; 7(2):106-13. PMID: 1707189.
    Citations: 2     Fields:    Translation:AnimalsCells
  130. Aronin N, Chase K, Sagar SM, Sharp FR, DiFiglia M. N-methyl-D-aspartate receptor activation in the neostriatum increases c-fos and fos-related antigens selectively in medium-sized neurons. Neuroscience. 1991; 44(2):409-20. PMID: 1834961.
    Citations: 8     Fields:    Translation:AnimalsCells
  131. DiFiglia M, Roberts RC, Benowitz LI. Immunoreactive GAP-43 in the neuropil of adult rat neostriatum: localization in unmyelinated fibers, axon terminals, and dendritic spines. J Comp Neurol. 1990 Dec 22; 302(4):992-1001. PMID: 2150524.
    Citations: 5     Fields:    Translation:AnimalsCells
  132. Roberts RC, DiFiglia M. Long-term survival of GABA-, enkephalin-, NADPH-diaphorase- and calbindin-d28k-containing neurons in fetal striatal grafts. Brain Res. 1990 Nov 05; 532(1-2):151-9. PMID: 1980852.
    Citations: 1     Fields:    Translation:AnimalsCells
  133. Chiocca EA, Choi BB, Cai WZ, DeLuca NA, Schaffer PA, DiFiglia M, Breakefield XO, Martuza RL. Transfer and expression of the lacZ gene in rat brain neurons mediated by herpes simplex virus mutants. New Biol. 1990 Aug; 2(8):739-46. PMID: 2178004.
    Citations: 26     Fields:    Translation:AnimalsCells
  134. DiFiglia M. Excitotoxic injury of the neostriatum: a model for Huntington's disease. Trends Neurosci. 1990 Jul; 13(7):286-9. PMID: 1695405.
    Citations: 99     Fields:    Translation:Animals
  135. Koroshetz WJ, Freese A, DiFiglia M. The correlation between excitatory amino acid-induced current responses and excitotoxicity in striatal cultures. Brain Res. 1990 Jun 25; 521(1-2):265-72. PMID: 1976414.
    Citations: 2     Fields:    Translation:AnimalsCells
  136. Freese A, DiFiglia M, Koroshetz WJ, Beal MF, Martin JB. Characterization and mechanism of glutamate neurotoxicity in primary striatal cultures. Brain Res. 1990 Jun 25; 521(1-2):254-64. PMID: 1976413.
    Citations: 10     Fields:    Translation:AnimalsCells
  137. Madras BK, Canfield DR, Pfaelzer C, Vittimberga FJ, Difiglia M, Aronin N, Bakthavachalam V, Baindur N, Neumeyer JL. Fluorescent and biotin probes for dopamine receptors: D1 and D2 receptor affinity and selectivity. Mol Pharmacol. 1990 Jun; 37(6):833-9. PMID: 2141665.
    Citations: 7     Fields:    Translation:Animals
  138. Roberts RC, DiFiglia M. Evidence for synaptic proliferation, reorganization, and growth in the excitotoxic lesioned adult rat caudate nucleus. Exp Neurol. 1990 Jan; 107(1):1-10. PMID: 2136832.
    Citations: 1     Fields:    Translation:AnimalsCells
  139. DiFiglia M, Aronin N. Synaptic interactions between GABAergic neurons and trigeminothalamic cells in the rat trigeminal nucleus caudalis. Synapse. 1990; 6(4):358-63. PMID: 1705057.
    Citations: 3     Fields:    Translation:AnimalsCells
  140. DiFiglia M, Marshall P, Covault J, Yamamoto M. Ultrastructural localization of molecular subtypes of immunoreactive neural cell adhesion molecule (NCAM) in the adult rodent striatum. J Neurosci. 1989 Dec; 9(12):4158-68. PMID: 2592994.
    Citations: 8     Fields:    Translation:AnimalsCells
  141. DiFiglia M, Christakos S, Aronin N. Ultrastructural localization of immunoreactive calbindin-D28k in the rat and monkey basal ganglia, including subcellular distribution with colloidal gold labeling. J Comp Neurol. 1989 Jan 22; 279(4):653-65. PMID: 2918090.
    Citations: 8     Fields:    Translation:HumansAnimals
  142. Roberts RC, DiFiglia M. Short- and long-term survival of large neurons in the excitotoxic lesioned rat caudate nucleus: a light and electron microscopic study. Synapse. 1989; 3(4):363-71. PMID: 2525824.
    Citations: 4     Fields:    Translation:AnimalsCells
  143. Difiglia M, Rafols JA. Synaptic organization of the globus pallidus. J Electron Microsc Tech. 1988 Nov; 10(3):247-63. PMID: 2906997.
    Citations: 8     Fields:    Translation:AnimalsCells
  144. Roberts RC, Difiglia M. Localization of immunoreactive GABA and enkephalin and NADPH-diaphorase-positive neurons in fetal striatal grafts in the quinolinic-acid-lesioned rat neostriatum. J Comp Neurol. 1988 Aug 15; 274(3):406-21. PMID: 2975675.
    Citations: 12     Fields:    Translation:AnimalsCells
  145. DiFiglia M, Schiff L, Deckel AW. Neuronal organization of fetal striatal grafts in kainate- and sham-lesioned rat caudate nucleus: light- and electron-microscopic observations. J Neurosci. 1988 Apr; 8(4):1112-30. PMID: 3357012.
    Citations: 5     Fields:    Translation:AnimalsCells
  146. Rafols JA, Aronin N, Difiglia M. A Golgi study of the monkey paraventricular nucleus: neuronal types, afferent and efferent fibers. J Comp Neurol. 1987 Mar 22; 257(4):595-613. PMID: 2447130.
    Citations: 2     Fields:    Translation:AnimalsCells
  147. DiFiglia M. Synaptic organization of cholinergic neurons in the monkey neostriatum. J Comp Neurol. 1987 Jan 08; 255(2):245-58. PMID: 3819015.
    Citations: 25     Fields:    Translation:AnimalsCells
  148. Difiglia M, Graveland GA, Schiff L. Cytochrome oxidase activity in the rat caudate nucleus: light and electron microscopic observations. J Comp Neurol. 1987 Jan 01; 255(1):137-45. PMID: 3029185.
    Citations:    Fields:    Translation:AnimalsCells
  149. Aronin N, Chase K, DiFiglia M. Glutamic acid decarboxylase and enkephalin immunoreactive axon terminals in the rat neostriatum synapse with striatonigral neurons. Brain Res. 1986 Feb 12; 365(1):151-8. PMID: 3512036.
    Citations: 4     Fields:    Translation:AnimalsCells
  150. DiFiglia M, Carey J. Large neurons in the primate neostriatum examined with the combined Golgi-electron microscopic method. J Comp Neurol. 1986 Feb 01; 244(1):36-52. PMID: 3950089.
    Citations: 12     Fields:    Translation:AnimalsCells
  151. Graveland GA, Williams RS, DiFiglia M. A Golgi study of the human neostriatum: neurons and afferent fibers. J Comp Neurol. 1985 Apr 15; 234(3):317-33. PMID: 3988987.
    Citations: 31     Fields:    Translation:HumansCells
  152. Graveland GA, DiFiglia M. The frequency and distribution of medium-sized neurons with indented nuclei in the primate and rodent neostriatum. Brain Res. 1985 Feb 18; 327(1-2):307-11. PMID: 3986508.
    Citations: 102     Fields:    Translation:AnimalsCells
  153. Graveland GA, Williams RS, DiFiglia M. Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science. 1985 Feb 15; 227(4688):770-3. PMID: 3155875.
    Citations: 178     Fields:    Translation:HumansCells
  154. DiFiglia M, Aronin N. Quantitative electron microscopic study of immunoreactive somatostatin axons in the rat neostriatum. Neurosci Lett. 1984 Sep 07; 50(1-3):325-31. PMID: 6149504.
    Citations:    Fields:    Translation:Animals
  155. Aronin N, Difiglia M, Graveland GA, Schwartz WJ, Wu JY. Localization of immunoreactive enkephalins in GABA synthesizing neurons of the rat neostriatum. Brain Res. 1984 May 23; 300(2):376-80. PMID: 6375810.
    Citations: 20     Fields:    Translation:Animals
  156. DiFiglia M, Aronin N. Immunoreactive leu-enkephalin in the monkey hypothalamus including observations on its ultrastructural localization in the paraventricular nucleus. J Comp Neurol. 1984 May 20; 225(3):313-26. PMID: 6144702.
    Citations: 1     Fields:    Translation:AnimalsCells
  157. Difiglia M, Aronin N, Leeman SE. Ultrastructural localization of immunoreactive neurotensin in the monkey superficial dorsal horn. J Comp Neurol. 1984 May 01; 225(1):1-12. PMID: 6373845.
    Citations: 3     Fields:    Translation:AnimalsCells
  158. Maciewicz R, Phipps BS, Foote WE, Aronin N, DiFiglia M. The distribution of substance P-containing neurons in the cat Edinger-Westphal nucleus: relationship to efferent projection systems. Brain Res. 1983 Jul 04; 270(2):217-30. PMID: 6192874.
    Citations: 15     Fields:    Translation:Animals
  159. Difiglia M, Pasik P, Pasik T. A Golgi and ultrastructural study of the monkey globus pallidus. J Comp Neurol. 1982 Nov 20; 212(1):53-75. PMID: 7174908.
    Citations: 13     Fields:    Translation:AnimalsCells
  160. DiFiglia M, Aronin N. Ultrastructural features of immunoreactive somatostatin neurons in the rat caudate nucleus. J Neurosci. 1982 Sep; 2(9):1267-74. PMID: 6126530.
    Citations: 21     Fields:    Translation:AnimalsCells
  161. DiFiglia M, Aronin N, Leeman SE. Light microscopic and ultrastructural localization of immunoreactive substance P in the dorsal horn of monkey spinal cord. Neuroscience. 1982 May; 7(5):1127-39. PMID: 6180349.
    Citations: 8     Fields:    Translation:AnimalsCells
  162. DiFiglia M, Aronin N, Martin JB. Light and electron microscopic localization of immunoreactive Leu-enkephalin in the monkey basal ganglia. J Neurosci. 1982 Mar; 2(3):303-20. PMID: 6121017.
    Citations: 11     Fields:    Translation:AnimalsCells
  163. DiFiglia M, Aronin N, Leeman SE. Immunoreactive substance P in the substantia nigra of the monkey: light and electron microscopic localization. Brain Res. 1982 Feb 11; 233(2):381-8. PMID: 6174178.
    Citations: 3     Fields:    Translation:AnimalsCells
  164. Aronin N, DiFiglia M, Liotta AS, Martin JB. Ultrastructural localization and biochemical features of immunoreactive LEU-enkephalin in monkey dorsal horn. J Neurosci. 1981 Jun; 1(6):561-77. PMID: 6286896.
    Citations: 11     Fields:    Translation:AnimalsCells
  165. Difiglia M, Pasik T, Pasik P. Ultrastructure of Golgi-impregnated and gold-toned spiny and aspiny neurons in the monkey neostriatum. J Neurocytol. 1980 Aug; 9(4):471-92. PMID: 6160212.
    Citations: 17     Fields:    Translation:AnimalsCells
  166. Difiglia M, Pasik P, Pasik T. Early postnatal development of the monkey neostriatum: a Golgi and ultrastructural study. J Comp Neurol. 1980 Mar 15; 190(2):303-31. PMID: 6769982.
    Citations: 11     Fields:    Translation:AnimalsCells
  167. DiFiglia M, Pasik T, Pasik P. Developmental aspects of neostriatal organization in monkeys. Appl Neurophysiol. 1979; 42(1-2):81-3. PMID: 110259.
    Citations:    Fields:    Translation:AnimalsCells
  168. Difiglia M, Pasik T, Pasik P. A Golgi study of afferent fibers in the neostriatum of monkeys. Brain Res. 1978 Aug 25; 152(2):341-7. PMID: 98215.
    Citations: 3     Fields:    Translation:AnimalsCells
  169. DiFiglia M, Pasik P, Pasik T. A Golgi study of neuronal types in the neostriatum of monkeys. Brain Res. 1976 Sep 17; 114(2):245-56. PMID: 822916.
    Citations: 44     Fields:    Translation:AnimalsCells
  170. Pasik P, Pasik T, DiFiglia M. Quantitative aspects of neuronal organization in the neostriatum of the macaque monkey. Res Publ Assoc Res Nerv Ment Dis. 1976; 55:57-90. PMID: 827000.
    Citations: 5     Fields:    Translation:AnimalsCells
  171. London WT, DiFiglia M, Rodgers J. Failure of transplacental transmission of Australia antigen. Lancet. 1969 Oct 25; 2(7626):900. PMID: 4186466.
    Citations: 7     Fields:    Translation:HumansCells
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Funded by the NIH National Center for Advancing Translational Sciences through its Clinical and Translational Science Awards Program, grant number UL1TR002541.