研究项目和附属机构
研究兴趣
Mechanisms of brain malformations and retinal degeneration.
研究抽象
Mechanisms and therapeutic development in genetic retinal degeneration and brain malformations
Our laboratory studies the mechanisms of genetic diseases affecting the central nervous system, 包括眼睛和大脑, 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. 具体地说, 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. 在我们的实验室, we use mouse and zebrafish to model these diseases and to aid in the discovery of experimental therapeutics including gene therapy.
选定的出版物
刘毅，曹生，于敏， 胡H. TMEM216 Deletion Causes Mislocalization of Cone Opsin and Rhodopsin and Photoreceptor Degeneration in Zebrafish. 投资眼科视觉科学. 61(8):24. doi: 10.1167 / iovs.61.8.24, 2020年中期:32687549
Liu Y, Yu M, Shang X, Nguyen MHH, Balakrishnan S, Sager R, 胡H. Eyes shut homolog (EYS) interacts with matriglycan of O-mannosyl glycans whose deficiency results in EYS mislocalization and degeneration of photoreceptors. Sci代表. 10(1):7795. doi: 10.1038/s41598-020-64752-4, 2020 PMID: 32385361
Biswas S, Watters J, Bachay G, Varshney S, Hunter DD, 胡H，布伦肯WJ. Laminin-dystroglycan signaling regulates retinal arteriogenesis. 美国实验生物学学会联合会J. 2018年6月6日:fj201800232R. doi: 10.1096/fj.201800232R. [Epub ahead of print] PMID:29874128
胡H，刘毅，班波凯，何毅，于敏. Postnatal Gene Therapy Improves Spatial Learning Despite the Presence of 神经元al Ectopia in a Model of 神经元al Migration Disorder. 基因(巴塞尔). 2016年11月29日;7(12). pii: E105.PMID: 27916859
Bartels MF, Winterhalter PR, Yu J, Liu Y, Lommel M, Möhrlen F, 胡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. 《推荐最近最火的赌博软件》. 2016年11月3日;11(11):e0166119. doi: 10.1371 /杂志.玉米饼.0166119. eCollection 2016.PMID: 27812179
Yu M, Liu Y, Li J, Natale BN, Cao S, Wang D, Amack JD, 胡H. Eyes shut homolog is required for maintaining the ciliary pocket and survival of photoreceptors in zebrafish. 开放杂志. 2016年11月15日;5(11):1662-1673. doi: 10.1242 /生物.021584.PMID: 27737822
Halfter W, Oertle P, Monnier CA, Camenzind L, Reyes-Lua M, 胡H, Candiello J, Labilloy A, Balasubramani M, Henrich PB, Plodinec M. New concepts in basement membrane biology. 2月J. 2015年12月,282 (23):4466 - 79. doi: 10.1111 / 2月.13495. Epub 2015年9月21日. 审查.PMID: 26299746
Zhang P, Yang Y, Candiello J, Thorn TL, Gray N, Halfter WM, 胡H. Biochemical and biophysical changes underlie the mechanisms of basement membrane disruptions in a mouse model of dystroglycanopathy. 矩阵杂志. 2013年4月24日;32(3-4):196-207. doi: 10.1016/j.matbio.2013.02.002. 2013年2月27日.PMID: 23454088
Yu M, He Y, Wang K, Zhang P, Zhang S, 胡H. Adeno-associated viral-mediated LARGE gene therapy rescues the muscular dystrophic phenotype in mouse models of dystroglycanopathy. 哼，吉恩。. 2013年3月,24 (3):317 - 30. doi: 10.1089 /哼.2012.084.PMID: 23379513
张志，张鹏， 胡H. LARGE expression augments the glycosylation of glycoproteins in addition to α-dystroglycan conferring laminin binding. 《推荐最近最火的赌博软件》. 2011年4月20日;6(4):e19080. doi: 10.1371 /杂志.玉米饼.0019080.PMID: 21533062
胡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 .神经科. 2011年5月1日;519(7):1320-37. doi: 10.1002 / cne.22572.PMID: 21452199
胡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. 摩尔活力. 2010年7月28日;16:14 . 15-28.PMID: 20680099
胡H杨勇，叶德安，熊燕，齐勇. 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 .神经科. 2007年5月10日;502(2):168-83.PMID: 17479518
Yang Y, Zhang P, Xiong Y, Li X, Qi Y, 胡H. Ectopia of meningeal fibroblasts and reactive gliosis in the cerebral cortex of the mouse model of muscle-eye-brain disease. J .神经科. 2007年12月10日;05(5):459-77.PMID: 17924568
Liu J, Ball SL, Yang Y, Mei P, Zhang L, Shi H, Kaminski HJ, Lemmon VP, 胡H. A genetic model for muscle-eye-brain disease in mice lacking protein O-mannose 1,2-N-acetylglucosaminyltransferase (POMGnT1). 机械工程开发. 2006年3月,123 (3):228 - 40. 2006年2月3日.PMID: 16458488
胡H. Cell-surface heparan sulfate is involved in the repulsive guidance activities of Slit2 protein. Nat >. 2001年7月,4 (7):695 - 701.PMID: 11426225
胡H. Polysialic acid regulates chain formation by migrating olfactory interneuron precursors. 神经科学杂志. 2000年9月1日;61(5):480-92.PMID: 10956417
胡H. Chemorepulsion of neuronal migration by Slit2 in the developing mammalian forebrain. 神经元. 1999年8月,23(4):703 - 11所示.PMID: 10482237