Harvard Catalyst Profiles

Contact, publication, and social network information about Harvard faculty and fellows.

Marian DiFiglia, Ph.D.

Co-Author

This page shows the publications co-authored by Marian DiFiglia and Kimberly Kegel.
Connection Strength

4.252
  1. 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.
    View in: PubMed
    Score: 0.505
  2. 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.
    View in: PubMed
    Score: 0.456
  3. 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.
    View in: PubMed
    Score: 0.348
  4. 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.
    View in: PubMed
    Score: 0.270
  5. 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.
    View in: PubMed
    Score: 0.249
  6. 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.
    View in: PubMed
    Score: 0.203
  7. 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.
    View in: PubMed
    Score: 0.156
  8. 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.
    View in: PubMed
    Score: 0.145
  9. 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.
    View in: PubMed
    Score: 0.145
  10. 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.
    View in: PubMed
    Score: 0.139
  11. 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.
    View in: PubMed
    Score: 0.138
  12. 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.
    View in: PubMed
    Score: 0.137
  13. 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.
    View in: PubMed
    Score: 0.136
  14. 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.
    View in: PubMed
    Score: 0.120
  15. 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.
    View in: PubMed
    Score: 0.118
  16. 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.
    View in: PubMed
    Score: 0.116
  17. 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.
    View in: PubMed
    Score: 0.115
  18. 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.
    View in: PubMed
    Score: 0.114
  19. 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.
    View in: PubMed
    Score: 0.109
  20. 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.
    View in: PubMed
    Score: 0.090
  21. 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.
    View in: PubMed
    Score: 0.078
  22. 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.
    View in: PubMed
    Score: 0.077
  23. 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.
    View in: PubMed
    Score: 0.067
  24. 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.
    View in: PubMed
    Score: 0.064
  25. 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.
    View in: PubMed
    Score: 0.063
  26. 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.
    View in: PubMed
    Score: 0.035
  27. 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.
    View in: PubMed
    Score: 0.032
  28. 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.
    View in: PubMed
    Score: 0.029
Connection Strength
The connection strength for co-authors is the sum of the scores for each of their shared publications.

Publication scores are based on many factors, including how long ago they were written and whether the person is a first or senior author.
Funded by the NIH National Center for Advancing Translational Sciences through its Clinical and Translational Science Awards Program, grant number UL1TR002541.