Major Research Areas
Upstate boasts basic and clinical researchers with diverse expertise in neuroscience, molecular genetics, genomics, epigenetics, structural biology, infectious disease, and behavior disorders. This allows students the opportunity to perform research in a wide range of research areas and easily collaborate when new expertise is needed.
Huaiyu Hu, PhD
- Associate Professor of Neuroscience and Physiology
Research Programs and Affiliations
- Biomedical Sciences Program
- Neuroscience Program
- Neuroscience and Physiology
- Physiology Program
Molecular studies of brain malformations.
Mechanisms and therapeutic development in genetic retinal degeneration and brain malformations
Our laboratory studies the mechanisms of genetic diseases affecting the central nervous system, including the eye and the brain, and developing gene therapies for these genetic diseases. We are especially interested in how cell-extracellular matrix interactions are involved in the development of the brain and the retina and how disruptions of such interactions affect normal development. We focus on two types of genetic diseases, retinitis pigmentosa and congenital muscular dystrophies that also involve the central nervous system. Specifically, we study the effects of abnormal protein glycosylation on cell-extracellular matrix interactions and how defective cell-extracellular matrix interactions cause retinal dystrophy and brain dysfunction. In our laboratory, we use mouse and zebrafish to model these diseases and to aid in the discovery of experimental therapeutics including gene therapy.
Yu M, He Y, Wang K, Zhang P, Zhang S, Hu H. Adeno-associated viral-mediated LARGE gene therapy rescues the muscular dystrophic phenotype in mouse models of dystroglycanopathy. Hum Gene Ther. 2013 Mar;24(3):317-30. doi: 10.1089/hum.2012.084.PMID:23379513
Zhang Z, Zhang P, Hu H. LARGE expression augments the glycosylation of glycoproteins in addition to α-dystroglycan conferring laminin binding. PLoS One. 2011 Apr 20;6(4):e19080. doi: 10.1371/journal.pone.0019080.PMID:21533062
Hu H, Li J, Gagen CS, Gray NW, Zhang Z, Qi Y, Zhang P. Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development. J Comp Neurol. 2011 May 1;519(7):1320-37. doi: 10.1002/cne.22572.PMID:21452199
Hu H, Candiello J, Zhang P, Ball SL, Cameron DA, Halfter W. Retinal ectopias and mechanically weakened basement membrane in a mouse model of muscle-eye-brain (MEB) disease congenital muscular dystrophy. Mol Vis. 2010 Jul 28;16:1415-28.PMID:20680099
Hu H, Yang Y, Eade A, Xiong Y, Qi Y. Breaches of the pial basement membrane and disappearance of the glia limitans during development underlie the cortical lamination defect in the mouse model of muscle-eye-brain disease. J Comp Neurol. 2007 May 10;502(2):168-83.PMID:17479518
Yang Y, Zhang P, Xiong Y, Li X, Qi Y, Hu H. Ectopia of meningeal fibroblasts and reactive gliosis in the cerebral cortex of the mouse model of muscle-eye-brain disease. J Comp Neurol. 2007 Dec 10;505(5):459-77.PMID:17924568
Liu J, Ball SL, Yang Y, Mei P, Zhang L, Shi H, Kaminski HJ, Lemmon VP, Hu H. A genetic model for muscle-eye-brain disease in mice lacking protein O-mannose 1,2-N-acetylglucosaminyltransferase (POMGnT1). Mech Dev. 2006 Mar;123(3):228-40. Epub 2006 Feb 3.PMID:16458488
Hu H. Cell-surface heparan sulfate is involved in the repulsive guidance activities of Slit2 protein. Nat Neurosci. 2001 Jul;4(7):695-701.PMID:11426225
Hu H. Polysialic acid regulates chain formation by migrating olfactory interneuron precursors. J Neurosci Res. 2000 Sep 1;61(5):480-92.PMID:10956417
Hu H. Chemorepulsion of neuronal migration by Slit2 in the developing mammalian forebrain. Neuron. 1999 Aug;23(4):703-11.PMID:10482237