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Jeffrey R. Holt, Ph.D.

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Mentoring
Efficacy of Transfection of Inner and Outer Hair Cells Using Adeno-Associated Virus
Summer, 06/02/13 - 08/10/13

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. R13DC017050 (HOLT, JEFFREY R) Mar 15, 2018 - Aug 31, 2018
    NIH/NIDCD
    2018 Auditory System Gordon Research Conference & Gordon Research Seminar
    Role: Principal Investigator
  2. R01DC013521 (HOLT, JEFFREY R) Jul 1, 2013 - Jun 30, 2018
    NIH/NIDCD
    TMC gene function in sensory hair cells
    Role: Principal Investigator
  3. R21DC011720 (HOLT, JEFFREY R) Jul 1, 2011 - Jun 30, 2014
    NIH/NIDCD
    Inducible Expression of Exogenous Potassium Channels in Hair Cells
    Role: Principal Investigator
  4. R01DC005439 (HOLT, JEFFREY R) Sep 1, 2001 - Jul 31, 2020
    NIH/NIDCD
    Ion Channel Function in Auditory and Vestibular Hair Cells
    Role: Principal Investigator
  5. R56DC005439 (HOLT, JEFFREY R) Sep 1, 2001 - Jun 30, 2009
    NIH/NIDCD
    Ion Channel Function in Auditory &Vestibular Hair Cells
    Role: 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.
List All   |   Timeline
  1. Holt JR, Geleoc GS. Split otoferlins reunited. EMBO Mol Med. 2018 Dec 20. PMID: 30573543.
    View in: PubMed
  2. Corey DP, Akyuz N, Holt JR. Function and Dysfunction of TMC Channels in Inner Ear Hair Cells. Cold Spring Harb Perspect Med. 2018 Oct 05. PMID: 30291150.
    View in: PubMed
  3. Pan B, Akyuz N, Liu XP, Asai Y, Nist-Lund C, Kurima K, Derfler BH, György B, Limapichat W, Walujkar S, Wimalasena LN, Sotomayor M, Corey DP, Holt JR. TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells. Neuron. 2018 Aug 22; 99(4):736-753.e6. PMID: 30138589.
    View in: PubMed
  4. Asai Y, Pan B, Nist-Lund C, Galvin A, Lukashkin AN, Lukashkina VA, Chen T, Zhou W, Zhu H, Russell IJ, Holt JR, Géléoc GSG. Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1. Sci Rep. 2018 Aug 14; 8(1):12124. PMID: 30108254.
    View in: PubMed
  5. Nakanishi H, Kurima K, Pan B, Wangemann P, Fitzgerald TS, Géléoc GS, Holt JR, Griffith AJ. Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function. Sci Rep. 2018 Aug 14; 8(1):12125. PMID: 30108230.
    View in: PubMed
  6. Taylor RR, Filia A, Paredes U, Asai Y, Holt JR, Lovett M, Forge A. Regenerating hair cells in vestibular sensory epithelia from humans. Elife. 2018 07 18; 7. PMID: 30019672.
    View in: PubMed
  7. Gao X, Tao Y, Lamas V, Huang M, Yeh WH, Pan B, Hu YJ, Hu JH, Thompson DB, Shu Y, Li Y, Wang H, Yang S, Xu Q, Polley DB, Liberman MC, Kong WJ, Holt JR, Chen ZY, Liu DR. Treatment of autosomal dominant hearing loss by in vivo delivery of genome editing agents. Nature. 2018 01 11; 553(7687):217-221. PMID: 29258297.
    View in: PubMed
  8. Ahmed H, Shubina-Oleinik O, Holt JR. Emerging Gene Therapies for Genetic Hearing Loss. J Assoc Res Otolaryngol. 2017 Oct; 18(5):649-670. PMID: 28815315.
    View in: PubMed
  9. Koehler KR, Nie J, Longworth-Mills E, Liu XP, Lee J, Holt JR, Hashino E. Generation of inner ear organoids containing functional hair cells from human pluripotent stem cells. Nat Biotechnol. 2017 06; 35(6):583-589. PMID: 28459451.
    View in: PubMed
  10. Landegger LD, Pan B, Askew C, Wassmer SJ, Gluck SD, Galvin A, Taylor R, Forge A, Stankovic KM, Holt JR, Vandenberghe LH. A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear. Nat Biotechnol. 2017 03; 35(3):280-284. PMID: 28165475.
    View in: PubMed
  11. Pan B, Askew C, Galvin A, Heman-Ackah S, Asai Y, Indzhykulian AA, Jodelka FM, Hastings ML, Lentz JJ, Vandenberghe LH, Holt JR, Géléoc GS. Gene therapy restores auditory and vestibular function in a mouse model of Usher syndrome type 1c. Nat Biotechnol. 2017 03; 35(3):264-272. PMID: 28165476.
    View in: PubMed
  12. Corey DP, Holt JR. Are TMCs the Mechanotransduction Channels of Vertebrate Hair Cells? J Neurosci. 2016 10 26; 36(43):10921-10926. PMID: 27798174.
    View in: PubMed
  13. Shibata SB, Ranum PT, Moteki H, Pan B, Goodwin AT, Goodman SS, Abbas PJ, Holt JR, Smith RJH. RNA Interference Prevents Autosomal-Dominant Hearing Loss. Am J Hum Genet. 2016 06 02; 98(6):1101-1113. PMID: 27236922.
    View in: PubMed
  14. Liu XP, Koehler KR, Mikosz AM, Hashino E, Holt JR. Functional development of mechanosensitive hair cells in stem cell-derived organoids parallels native vestibular hair cells. Nat Commun. 2016 05 24; 7:11508. PMID: 27215798.
    View in: PubMed
  15. Akyuz N, Holt JR. Plug-N-Play: Mechanotransduction Goes Modular. Neuron. 2016 Mar 16; 89(6):1128-1130. PMID: 26985721.
    View in: PubMed
  16. Imtiaz A, Maqsood A, Rehman AU, Morell RJ, Holt JR, Friedman TB, Naz S. Recessive mutations of TMC1 associated with moderate to severe hearing loss. Neurogenetics. 2016 Apr; 17(2):115-123. PMID: 26879195; PMCID: PMC4795972 [Available on 04/01/17].
  17. Kurima K, Ebrahim S, Pan B, Sedlacek M, Sengupta P, Millis BA, Cui R, Nakanishi H, Fujikawa T, Kawashima Y, Choi BY, Monahan K, Holt JR, Griffith AJ, Kachar B. TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia. Cell Rep. 2015 Sep 08; 12(10):1606-17. PMID: 26321635.
    View in: PubMed
  18. Pan B, Holt JR. The molecules that mediate sensory transduction in the mammalian inner ear. Curr Opin Neurobiol. 2015 Oct; 34:165-71. PMID: 26218316; PMCID: PMC4698977 [Available on 10/01/16].
  19. Askew C, Rochat C, Pan B, Asai Y, Ahmed H, Child E, Schneider BL, Aebischer P, Holt JR. Tmc gene therapy restores auditory function in deaf mice. Sci Transl Med. 2015 Jul 08; 7(295):295ra108. PMID: 26157030.
    View in: PubMed
  20. Kawashima Y, Kurima K, Pan B, Griffith AJ, Holt JR. Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation. Pflugers Arch. 2015 Jan; 467(1):85-94. PMID: 25074487.
    View in: PubMed
  21. Géléoc GS, Holt JR. Sound strategies for hearing restoration. Science. 2014 May 09; 344(6184):1241062. PMID: 24812404; PMCID: PMC4148779.
  22. Horwitz GC, Risner-Janiczek JR, Holt JR. Mechanotransduction and hyperpolarization-activated currents contribute to spontaneous activity in mouse vestibular ganglion neurons. J Gen Physiol. 2014 Apr; 143(4):481-97. PMID: 24638995; PMCID: PMC3971655.
  23. Holt JR, Pan B, Koussa MA, Asai Y. TMC function in hair cell transduction. Hear Res. 2014 May; 311:17-24. PMID: 24423408; PMCID: PMC4096076.
  24. Zou J, Zheng T, Ren C, Askew C, Liu XP, Pan B, Holt JR, Wang Y, Yang J. Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in mice. Hum Mol Genet. 2014 May 01; 23(9):2374-90. PMID: 24334608; PMCID: PMC3976334.
  25. Yu WM, Appler JM, Kim YH, Nishitani AM, Holt JR, Goodrich LV. A Gata3-Mafb transcriptional network directs post-synaptic differentiation in synapses specialized for hearing. Elife. 2013 Dec 10; 2:e01341. PMID: 24327562; PMCID: PMC3851837.
  26. Kim YH, Holt JR. Functional contributions of HCN channels in the primary auditory neurons of the mouse inner ear. J Gen Physiol. 2013 Sep; 142(3):207-23. PMID: 23980193; PMCID: PMC3753603.
  27. Pan B, Géléoc GS, Asai Y, Horwitz GC, Kurima K, Ishikawa K, Kawashima Y, Griffith AJ, Holt JR. TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner ear. Neuron. 2013 Aug 07; 79(3):504-15. PMID: 23871232; PMCID: PMC3827726.
  28. Holt JR, Vandenberghe LH. Gene therapy for deaf mice goes viral. Mol Ther. 2012 Oct; 20(10):1836-7. PMID: 23023054; PMCID: PMC3464630.
  29. Geng R, Melki S, Chen DH, Tian G, Furness DN, Oshima-Takago T, Neef J, Moser T, Askew C, Horwitz G, Holt JR, Imanishi Y, Alagramam KN. The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene. J Neurosci. 2012 Jul 11; 32(28):9485-98. PMID: 22787034; PMCID: PMC3422646.
  30. Levin ME, Holt JR. The function and molecular identity of inward rectifier channels in vestibular hair cells of the mouse inner ear. J Neurophysiol. 2012 Jul; 108(1):175-86. PMID: 22496522; PMCID: PMC3434605.
  31. Kawashima Y, Géléoc GS, Kurima K, Labay V, Lelli A, Asai Y, Makishima T, Wu DK, Della Santina CC, Holt JR, Griffith AJ. Mechanotransduction in mouse inner ear hair cells requires transmembrane channel-like genes. J Clin Invest. 2011 Dec; 121(12):4796-809. PMID: 22105175; PMCID: PMC3223072.
  32. Horwitz GC, Risner-Janiczek JR, Jones SM, Holt JR. HCN channels expressed in the inner ear are necessary for normal balance function. J Neurosci. 2011 Nov 16; 31(46):16814-25. PMID: 22090507; PMCID: PMC3477615.
  33. Steigelman KA, Lelli A, Wu X, Gao J, Lin S, Piontek K, Wodarczyk C, Boletta A, Kim H, Qian F, Germino G, Géléoc GS, Holt JR, Zuo J. Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells. J Neurosci. 2011 Aug 24; 31(34):12241-50. PMID: 21865467; PMCID: PMC3164988.
  34. Charizopoulou N, Lelli A, Schraders M, Ray K, Hildebrand MS, Ramesh A, Srisailapathy CR, Oostrik J, Admiraal RJ, Neely HR, Latoche JR, Smith RJ, Northup JK, Kremer H, Holt JR, Noben-Trauth K. Gipc3 mutations associated with audiogenic seizures and sensorineural hearing loss in mouse and human. Nat Commun. 2011 Feb 15; 2:201. PMID: 21326233; PMCID: PMC3105340.
  35. Lelli A, Kazmierczak P, Kawashima Y, Müller U, Holt JR. Development and regeneration of sensory transduction in auditory hair cells requires functional interaction between cadherin-23 and protocadherin-15. J Neurosci. 2010 Aug 25; 30(34):11259-69. PMID: 20739546; PMCID: PMC2949085.
  36. Horwitz GC, Lelli A, Géléoc GS, Holt JR. HCN channels are not required for mechanotransduction in sensory hair cells of the mouse inner ear. PLoS One. 2010 Jan 07; 5(1):e8627. PMID: 20062532; PMCID: PMC2797612.
  37. Asai Y, Holt JR, Géléoc GS. A quantitative analysis of the spatiotemporal pattern of transient receptor potential gene expression in the developing mouse cochlea. J Assoc Res Otolaryngol. 2010 Mar; 11(1):27-37. PMID: 19834762; PMCID: PMC2820207.
  38. Li H, Liu H, Corrales CE, Risner JR, Forrester J, Holt JR, Heller S, Edge AS. Differentiation of neurons from neural precursors generated in floating spheres from embryonic stem cells. BMC Neurosci. 2009 Sep 24; 10:122. PMID: 19778451; PMCID: PMC2761926.
  39. Lelli A, Asai Y, Forge A, Holt JR, Géléoc GS. Tonotopic gradient in the developmental acquisition of sensory transduction in outer hair cells of the mouse cochlea. J Neurophysiol. 2009 Jun; 101(6):2961-73. PMID: 19339464; PMCID: PMC2694104.
  40. Schwander M, Xiong W, Tokita J, Lelli A, Elledge HM, Kazmierczak P, Sczaniecka A, Kolatkar A, Wiltshire T, Kuhn P, Holt JR, Kachar B, Tarantino L, Müller U. A mouse model for nonsyndromic deafness (DFNB12) links hearing loss to defects in tip links of mechanosensory hair cells. Proc Natl Acad Sci U S A. 2009 Mar 31; 106(13):5252-7. PMID: 19270079; PMCID: PMC2664065.
  41. Collado MS, Holt JR. Can neurosphere production help restore inner ear transduction? Proc Natl Acad Sci U S A. 2009 Jan 06; 106(1):8-9. PMID: 19118205; PMCID: PMC2629198.
  42. Kesser BW, Hashisaki GT, Holt JR. Gene transfer in human vestibular epithelia and the prospects for inner ear gene therapy. Laryngoscope. 2008 May; 118(5):821-31. PMID: 18300702; PMCID: PMC2642479.
  43. Stauffer EA, Holt JR. Sensory transduction and adaptation in inner and outer hair cells of the mouse auditory system. J Neurophysiol. 2007 Dec; 98(6):3360-9. PMID: 17942617; PMCID: PMC2647849.
  44. Holt JR, Stauffer EA, Abraham D, Géléoc GS. Dominant-negative inhibition of M-like potassium conductances in hair cells of the mouse inner ear. J Neurosci. 2007 Aug 15; 27(33):8940-51. PMID: 17699675; PMCID: PMC2647843.
  45. Oshima K, Grimm CM, Corrales CE, Senn P, Martinez Monedero R, Géléoc GS, Edge A, Holt JR, Heller S. Differential distribution of stem cells in the auditory and vestibular organs of the inner ear. J Assoc Res Otolaryngol. 2007 Mar; 8(1):18-31. PMID: 17171473; PMCID: PMC2538418.
  46. Risner JR, Holt JR. Heterogeneous potassium conductances contribute to the diverse firing properties of postnatal mouse vestibular ganglion neurons. J Neurophysiol. 2006 Nov; 96(5):2364-76. PMID: 16855108; PMCID: PMC2638094.
  47. McGee J, Goodyear RJ, McMillan DR, Stauffer EA, Holt JR, Locke KG, Birch DG, Legan PK, White PC, Walsh EJ, Richardson GP. The very large G-protein-coupled receptor VLGR1: a component of the ankle link complex required for the normal development of auditory hair bundles. J Neurosci. 2006 Jun 14; 26(24):6543-53. PMID: 16775142; PMCID: PMC2682555.
  48. Senften M, Schwander M, Kazmierczak P, Lillo C, Shin JB, Hasson T, Géléoc GS, Gillespie PG, Williams D, Holt JR, Müller U. Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells. J Neurosci. 2006 Feb 15; 26(7):2060-71. PMID: 16481439; PMCID: PMC2712835.
  49. Stauffer EA, Scarborough JD, Hirono M, Miller ED, Shah K, Mercer JA, Holt JR, Gillespie PG. Fast adaptation in vestibular hair cells requires myosin-1c activity. Neuron. 2005 Aug 18; 47(4):541-53. PMID: 16102537; PMCID: PMC2682556.
  50. Géléoc GS, Risner JR, Holt JR. Developmental acquisition of voltage-dependent conductances and sensory signaling in hair cells of the embryonic mouse inner ear. J Neurosci. 2004 Dec 08; 24(49):11148-59. PMID: 15590931; PMCID: PMC2638092.
  51. Corey DP, García-Añoveros J, Holt JR, Kwan KY, Lin SY, Vollrath MA, Amalfitano A, Cheung EL, Derfler BH, Duggan A, Géléoc GS, Gray PA, Hoffman MP, Rehm HL, Tamasauskas D, Zhang DS. TRPA1 is a candidate for the mechanosensitive transduction channel of vertebrate hair cells. Nature. 2004 Dec 09; 432(7018):723-30. PMID: 15483558.
    View in: PubMed
  52. Géléoc GS, Holt JR. Developmental acquisition of sensory transduction in hair cells of the mouse inner ear. Nat Neurosci. 2003 Oct; 6(10):1019-20. PMID: 12973354; PMCID: PMC2669437.
  53. Colclasure JC, Holt JR. Transduction and adaptation in sensory hair cells of the mammalian vestibular system. Gravit Space Biol Bull. 2003 Jun; 16(2):61-70. PMID: 12959133.
    View in: PubMed
  54. Géléoc GS, Holt JR. Auditory amplification: outer hair cells pres the issue. Trends Neurosci. 2003 Mar; 26(3):115-7. PMID: 12591210.
    View in: PubMed
  55. Holt JR. Viral-mediated gene transfer to study the molecular physiology of the Mammalian inner ear. Audiol Neurootol. 2002 May-Jun; 7(3):157-60. PMID: 12053137.
    View in: PubMed
  56. Dumont RA, Zhao YD, Holt JR, Bähler M, Gillespie PG. Myosin-I isozymes in neonatal rodent auditory and vestibular epithelia. J Assoc Res Otolaryngol. 2002 Dec; 3(4):375-89. PMID: 12486594; PMCID: PMC3202438.
  57. Holt JR, Gillespie SK, Provance DW, Shah K, Shokat KM, Corey DP, Mercer JA, Gillespie PG. A chemical-genetic strategy implicates myosin-1c in adaptation by hair cells. Cell. 2002 Feb 08; 108(3):371-81. PMID: 11853671.
    View in: PubMed
  58. Holt JR, Corey DP. Two mechanisms for transducer adaptation in vertebrate hair cells. Proc Natl Acad Sci U S A. 2000 Oct 24; 97(22):11730-5. PMID: 11050202; PMCID: PMC34342.
  59. Holt JR, Vollrath MA, Eatock RA. Stimulus processing by type II hair cells in the mouse utricle. Ann N Y Acad Sci. 1999 May 28; 871:15-26. PMID: 10372060.
    View in: PubMed
  60. Holt JR. Biology in pictures. A sense of colour. Curr Biol. 1999 May 20; 9(10):R351. PMID: 10339436.
    View in: PubMed
  61. Holt JR, Johns DC, Wang S, Chen ZY, Dunn RJ, Marban E, Corey DP. Functional expression of exogenous proteins in mammalian sensory hair cells infected with adenoviral vectors. J Neurophysiol. 1999 Apr; 81(4):1881-8. PMID: 10200223.
    View in: PubMed
  62. Holt JR, Corey DP. Ion channel defects in hereditary hearing loss. Neuron. 1999 Feb; 22(2):217-9. PMID: 10069328.
    View in: PubMed
  63. Holt JR, Corey DP, Eatock RA. Mechanoelectrical transduction and adaptation in hair cells of the mouse utricle, a low-frequency vestibular organ. J Neurosci. 1997 Nov 15; 17(22):8739-48. PMID: 9348343.
    View in: PubMed
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Funded by the NIH/NCATS Clinical and Translational Science Award (CTSA) program, grant number UL1TR001102, and through institutional support from Harvard University, Harvard Medical School, Harvard T.H. Chan School of Public Health, Beth Israel Deaconess Medical Center, Boston Children's Hospital, Brigham and Women's Hospital, Massachusetts General Hospital and the Dana Farber Cancer Institute.