Huda Y. Zoghbi, M.D. |
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Huda Y. Zoghbi is an investigator at the Howard Hughes Medical Institute, and a professor of Pediatrics at Baylor College of Medicine, Houston where she also is a professor of Molecular and Human Genetics, Neurology, Neuroscience, and faculty in the Developmental Biology, Cell and Molecular Biology, and Translational Biology and Molecular Medicine Graduate Programs. A specialist in the study of development and neurogenetics, her work has encompassed many neurological disorders including the inherited degenerative balance disorders (spinocerebellar ataxias) and autism spectrum disorders such as Rett syndrome. The lab also investigates key and basic developmental questions about neuronal differentiation and development of the auditory and proprioceptive sensory system. |
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In a bottom-up approach, the Zoghbi lab identified the Math1 gene (mouse atonal homolog 1) and demonstrated its critical role for development of the auditory system (including inner ear hair cells), sensory balance (proprioception and vestibular system), respiratory control, and intestinal development. These basic developmental studies are beginning to provide insight about many human disorders (Figure 2).
For the first 15 years of her career, Dr. Zoghbi spent 20 percent of her time seeing patients with childhood neurological disorders. Driven by a desire to improve the clinical outcome of her patients, she became convinced that more basic research is needed. In more recent years, as her research efforts expanded to understand the complex disease mechanisms, her clinical activities became limited with a focus on clinical research protocols that enroll selected patients. Dr. Zoghbi participates in teaching in the various graduate and clinical programs to which she belongs. Dr. Zoghbi’s favorite part of her job is interacting with graduate students, fellows, and other trainees in the lab. Dr. Zoghbi cherishes her collaborations especially those long-standing ones with Harry T. Orr, Hugo Bellen, Juan Botas and more recently Nat Heintz. |
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Education: Dr. Zoghbi was born in Beirut, Lebanon where she attended the American University of Beirut for her undergraduate studies. She began medical school at AUB only to have her plans interrupted during her sophomore year when she was visiting family in the U.S. and could not return to Lebanon due to an escalating civil war. She transferred to Meharry Medical College where she graduated in 1979. Dr. Zoghbi then came to Baylor College of Medicine where she completed residency training in pediatrics and neurology and encountered her first Rett syndrome patient as a resident in 1983. Her patients, particularly those with Rett syndrome, inspired her to pursue research training in molecular genetics. She trained with Dr. Arthur L. Beaudet in the Department of Molecular and Human Genetics at Baylor College of Medicine. Upon completion of her training in 1988, she joined the faculty of Baylor College of Medicine as an assistant professor. |
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Selected Honors: 2009: International Rett Syndrome Foundation's Circle of Angels Research Award |
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Personal: Dr. Huda Zoghbi is married to Dr. William Zoghbi, Professor of Cardiology, DeBakey Heart Center, at the Methodist Hospital, and is the proud mother of Roula Zoghbi (Middlebury College, class 2007) and Anthony Zoghbi (Washington University in St. Louis, Class of 2009). She is most grateful to her husband and children for unconditional love and support; her mentors for setting her on the right track; her friends for embodying what friendship is; her patients for their trust and participation; and her past and current lab members for their tireless efforts and for making the lab a fun and stimulating place. Dr. Zoghbi enjoys reading, working out, gourmet cooking (especially when she teams up with her daughter, a superb dessert chef), and the opera. |
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Selected key publications: Lin X, Antalffy B, Kang D, Orr HT, Zoghbi HY. Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1. Nat Neurosci. 2000 Feb;3(2):157-63. Fernandez-Funez P, Rosales MLN, de Gouyon B, She W-C, Luchack J, Turiegano E, Benito J, Capovilla M, Skinner PJ, McCall A, Canal I, Orr HT, Zoghbi HY, Botas J. Identification of genes that modify ataxin-1 induced neurodegeneration. Nature. 2000 Nov 2;408(6808):101-6. Bermingham NA, Hassan B, Fernandez M, Banfi S, Bellen H, Fritzsch B, Zoghbi HY. Proprioceptor pathway development is dependent on Math1. Neuron. 2001 May;30(2):411-22. Cummings C J, Sun Y, Opal P, Antalffy B, Mestril R, Orr HT, Dillmann WH, Zoghbi HY. Over-expression of inducible HSP70 Chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum Mol Genet. 2001 Jul 1;10(14):1511-8. Yang Q, Bermingham N, Finegold M, Zoghbi H. Requirement of Math1 for secretory cell lineage commitment in the mouse intestine. Science. 2001 Dec 7;294(5549):2155-8. Watase K, Weeber EJ, Xu B, Antalffy B, Nellis A, Yuva-Paylor L, Armstrong D, Sweatt JD, Orr HT, Paylor RL Zoghbi HY. A long CAG repeat in the mouse Sca1 locus replicates human SCA1 features and reveals the impact of protein solubility on selective neurodegeneration. Neuron. 2002 Jun 13;34(6):905-19. Shahbazian MD, Young JI, Yuva-Paylor LA, Antalffy BA, Spencer CM, Noebels JL, Armstrong DL, Paylor R, Zoghbi HY. Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3. Neuron. 2002 Jul 18;35(2):243-54. Yoo S-Y, Pennesi ME, Weeber EJ, Xu B, Atkinson R, Chen S, Wu SM, Sweatt JD, Zoghbi HY. SCA7 knockin mice model human SCA7 and reveal gradual accumulation of ataxin-7 neurons and abnormalities in short-term plasticity. Neuron. 2003 Feb 6;37(3):383-401. Chen H-K, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EMC, Orr HT, Botas J, Zoghbi HY. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell. 2003 May 16;113(4):457-68. Collins AL, Levenson JM, Vilaythong AP, Richman R, Armstrong DL, Noebels JL, Sweatt JD, Zoghbi HY. Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet. 2004 Nov 1;13(21):2679-89. Tsuda H, Jafar-Nejad H, Patel AJ, Sun Y, Chen HK, Rose MF, Venken KJ, Botas J, Orr HT, Bellen HJ, Zoghbi HY. The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell. 2005 Aug 26;122(4):633-44. Tsuda H, Jafar-Nejad H, Patel AJ, Sun Y, Chen HK, Rose MF, Venken KJ, Botas J, Orr HT, Bellen HJ, Zoghbi HY. The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell. 2005 Aug 26;122(4):633-44. Wang VY, Rose MF, Zoghbi HY. Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum. Neuron. 2005 Oct 6;48(1):31-43. Young JI, Hong EP, Castle J, Crespo-Barreto J, Bowman AB, Rose MF, Kang D, Richman R, Johnson J, Berget S, Zoghbi H. Regulation of RNA splicing by the methylation-dependent transcriptional repressor MeCP2. Proc Natl Acad Sci U S A. 2005 Dec 6;102(49):17551-8. Moretti P, Levenson JM, Battaglia F, Atkinson R, Teague R, Antalffy B, Armstrong D, Arancio O, Sweatt JD, Zoghbi HY. Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome. J Neurosci. 2006 Jan 4;26(1):319-27. McGill BE, Bundle SF, Yaylaoglu MB, Carson JP, Thaller C, Zoghbi HY. Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A. 2006 Nov 28;103(48):18267-72. Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, Zoghbi HY. ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell. 2006 Dec 29;127(7):1335-47. Bowman AB, Lam YC, Jafar-Nejad P, Chen HK, Richman R, Samaco RC, Fryer JD, Kahle JJ, Orr HT, Zoghbi HY. Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes. Nat Genet. 2007 Mar;39(3):373-379. Chao HT, Zoghbi HY, Rosenmund C. MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron. 2007 Oct 4;56(1):58-65. PMID: 17920015 Chahrour M, Zoghbi HY.The story of Rett syndrome: from clinic to neurobiology. Neuron. 2007 Nov 8;56(3):422-37. Review. PMID: 17988628 Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, Zoghbi HY. Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature. 2008 Apr 10; 452(7188):713-8. Epub 2008 Mar 12. PMID: 18337722 Chahrour M, Jung SY, Shaw C, Zhou X, Wong ST, Qin J, Zoghbi HY. MeCP2, a key contributor to neurological disease, activates and represses transcription. Science. 2008 May 30;320(5880):1224-9. PMID: 18511691 PMCID: 2443785 Lee Y, Samaco RC, Gatchel JR, Thaller C, Orr HT, Zoghbi HY. miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis. Nat Neurosci. 2008 Oct;11(10):1137-9. Epub 2008 Aug 31. PMID: 18758459 PMCID: 2574629 Fyffe SL, Neul JL, Samaco RC, Chao HT, Ben-Shachar S, Moretti P, McGill BE, Goulding EH, Sullivan E, Tecott LH, Zoghbi HY. Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress. Neuron. 2008 Sep 25;59(6):947-58. PMID: 18817733 PMCID: 2597031 Ramocki MB, Zoghbi HY. Failure of neuronal homeostasis results in common neuropsychiatric phenotypes. Nature. 2008 Oct 16;455(7215):912-8. Review. PMID: 18923513 PMCID: 2696622 Zoghbi HY. Rett syndrome: what do we know for sure? Nat Neurosci. 2009 Mar;12(3):239-40. No abstract available. PMID: 19238181 Maricich SM, Wellnitz SA, Nelson AM, Lesniak DR, Gerling GJ, Lumpkin EA, Zoghbi HY. Merkel cells are essential for light-touch responses. Science. 2009 Jun 19;324(5934):1580-2.PMID: 19541997 PMCID: 2743005
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