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Welcome to the Zoghbi Laboratory website!

Dr. Huda Y. Zoghbi is a professor in the departments of Pediatrics, Molecular and Human Genetics, Neurology and Neuroscience at Baylor College of Medicine. She is also an investigator at the Howard Hughes Medical Institute and the director of the Jan & Dan Duncan Neurological Research Institute (NRI) at Texas Children's Hospital.

The laboratory uses genetic, biochemical, and cell biological approaches to explore the pathogenesis of polyglutamine neurodegenerative diseases and Rett syndrome, and to study genes essential for normal neurodevelopment.

Please visit the site to learn about ongoing research, peruse the publications, and get to know the lab members.



Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1 pp221 – 226 HT Orr, M-y Chung, S Banfi, TJ Kwiatkowski Jr., A Servadio, AL Beaudet, AE McCall, LA Duvick, LPW Ranum & HY Zoghbi

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat p937  EN Burright, HB Clark, A Servadio, T Matilla, RM Feddersen, WS Yunis, LA Duvick, HY Zoghbi, HT Orr N Ben-Arie, AE McCall, S Berkman, G Eichele, HJ Bellen, and HY Zoghbi.  Evolutionary Conservation of Sequence and Expression of the bHLH Protein Atonal Suggests a Conserved Role in Neurogenesis.  Hum. Mol. Genet. (1996) 5(9): 1207-1216 Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Klement IA, Skinner PJ, Kaytor MD, Yi H, Hersch SM, Clark HB, Zoghbi HY, Orr HT. Cell. 1998 Oct 2;95(1):41-53. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Cummings CJ, Mancini MA, Antalffy B, DeFranco DB, Orr HT, Zoghbi HY. Nat Genet. 1998 Jun;19(2):148-54. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2 pp185 – 188 RE Amir, IB. Van den Veyver, M Wan, CQ Tran, U Francke & HY Zoghbi Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice.  Cummings CJ, Sun Y, Opal P, Antalffy B, Mestril R, Orr HT, Dillmann WH, Zoghbi HY. Hum Mol Genet. 2001 Jul 1;10(14):1511-8.
Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Emamian ES, Kaytor MD, Duvick LA, Zu T, Tousey SK, Zoghbi HY, Clark HB, Orr HT. Neuron. 2003 May 8;38(3):375-87. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EM, Orr HT, Botas J, Zoghbi HY. Cell. 2003 May 16;113(4):457-68. Neuroscience: 2010 KAVLI PRIZE SYMPOSIUM Protein interactome reveals converging molecular pathways among autism disorders. Sakai Y, Shaw CA, Dawson BC, Dugas DV, Al-Mohtaseb Z, Hill DE, Zoghbi HY. Sci Transl Med. 2011 Jun 8;3(86):86ra49. Atoh1 governs the migration of postmitotic neurons that shape respiratory effectiveness at birth and chemoresponsiveness in adulthood. Huang WH, Tupal S, Huang TW, Ward CS, Neul JL, Klisch TJ, Gray PA, Zoghbi HY. Neuron. 2012 Sep 6;75(5):799-809. Overexpression of methyl-CpG binding protein 2 impairs T(H)1 responses. Yang T, Ramocki MB, Neul JL, Lu W, Roberts L, Knight J, Ward CS, Zoghbi HY, Kheradmand F, Corry DB. Sci Transl Med. 2012 Dec 5;4(163):163ra158. Human-specific regulation of MeCP2 levels in fetal brains by microRNA miR-483-5p. Han K, Gennarino VA, Lee Y, Pang K, Hashimoto-Torii K, Choufani S, Raju CS, Oldham MC, Weksberg R, Rakic P, Liu Z, Zoghbi HY. Genes Dev. 2013 Mar 1;27(5):485-90.

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Molecular and Human Genetics, Zoghbi laboratory
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Zoghbi lab

Last Modified: September 2012