Huda Y. Zoghbi

Baylor College of Medicine

Election Year: 2004
Primary Section: 24, Cellular and Molecular Neuroscience
Secondary Section: 41, Medical Genetics, Hematology, and Oncology
Membership Type: Member

Research Interests

My research centers on the nervous system, in development and disease. One branch of my lab is unraveling the molecular pathogenesis of spinocerebellar ataxias that are caused by a polyglutamine repeat expansion in the disease-causing proteins that interferes with protein folding and degradation; in both fly and mouse models of spinocerebellar ataxia type 1 (SCA1), we have shown that increasing chaperone activity can mitigate the neurodegenerative phenotype. We are currently studying mouse models of several different SCAs in hopes of gaining insight into the molecular ramifications of protein accumulation and identifying molecular targets that could lead to safe therapies. My lab also studies Math1 (mouse atonal homologue 1), which governs the development of many components of the proprioceptive pathway. We are trying to identify Math1's downstream targets to better grasp how one gene can control the fate of such divergent cell types as cerebellar granule neurons, inner ear hair cells, and intestinal secretory cells. Finally, we are investigating how mutations in the transcriptional repressor MeCP2 cause Rett syndrome and autistic spectrum disorders. We are using mouse models that either lack or over-express MeCP2 to identify MeCP2 targets and determine how MeCP2 influences synaptic plasticity, allowing the acquisition of motor, language, and social skills.

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