Huaiyu Hu profile picture
315 464-8143

Huaiyu Hu, PhD

4710 Institute For Human Performance (IHP)
505 Irving Avenue
Syracuse, NY 13210
Huaiyu Hu's email address generated as an image

CURRENT APPOINTMENTS

LANGUAGES

English

RESEARCH PROGRAMS AND AFFILIATIONS

Biomedical Sciences Program
Cancer Research Program
Neuroscience and Physiology
Neuroscience Program
Physiology Program

RESEARCH INTERESTS

Mechanisms of brain malformations and retinal degeneration.  

RESEARCH ABSTRACT

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.

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Selected Publications

Liu Y, Cao S, Yu M, Hu H. TMEM216 Deletion Causes Mislocalization of Cone Opsin and Rhodopsin and Photoreceptor Degeneration in Zebrafish. Invest Ophthalmol Vis Sci. 61(8):24. doi: 10.1167/iovs.61.8.24, 2020 PMID: 32687549  

Liu Y, Yu M, Shang X, Nguyen MHH, Balakrishnan S, Sager R, Hu H. Eyes shut homolog (EYS) interacts with matriglycan of O-mannosyl glycans whose deficiency results in EYS mislocalization and degeneration of photoreceptors. Sci Rep. 10(1):7795. doi: 10.1038/s41598-020-64752-4, 2020 PMID: 32385361

Biswas S, Watters J, Bachay G, Varshney S, Hunter DD, Hu H, Brunken WJ. Laminin-dystroglycan signaling regulates retinal arteriogenesis. FASEB J. 2018 Jun 6:fj201800232R. doi: 10.1096/fj.201800232R. [Epub ahead of print] PMID:29874128

Hu H, Liu Y, Bampoe K, He Y, Yu M. Postnatal Gene Therapy Improves Spatial Learning Despite the Presence of Neuronal Ectopia in a Model of Neuronal Migration Disorder. Genes (Basel). 2016 Nov 29;7(12). pii: E105.PMID:27916859

Bartels MF, Winterhalter PR, Yu J, Liu Y, Lommel M, Möhrlen F, Hu H, Feizi T, Westerlind U, Ruppert T, Strahl S. Protein O-Mannosylation in the Murine Brain: Occurrence of Mono-O-Mannosyl Glycans and Identification of New Substrates. PLoS One. 2016 Nov 3;11(11):e0166119. doi: 10.1371/journal.pone.0166119. eCollection 2016.PMID:27812179

Yu M, Liu Y, Li J, Natale BN, Cao S, Wang D, Amack JD, Hu H. Eyes shut homolog is required for maintaining the ciliary pocket and survival of photoreceptors in zebrafish. Biol Open. 2016 Nov 15;5(11):1662-1673. doi: 10.1242/bio.021584.PMID:27737822

Halfter W, Oertle P, Monnier CA, Camenzind L, Reyes-Lua M, Hu H, Candiello J, Labilloy A, Balasubramani M, Henrich PB, Plodinec M. New concepts in basement membrane biology. FEBS J. 2015 Dec;282(23):4466-79. doi: 10.1111/febs.13495. Epub 2015 Sep 21. Review.PMID:26299746

Zhang P, Yang Y, Candiello J, Thorn TL, Gray N, Halfter WM, Hu H. Biochemical and biophysical changes underlie the mechanisms of basement membrane disruptions in a mouse model of dystroglycanopathy. Matrix Biol. 2013 Apr 24;32(3-4):196-207. doi: 10.1016/j.matbio.2013.02.002. Epub 2013 Feb 27.PMID:23454088

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

 

PUBLICATIONS

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