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Richard Steet
Assistant Professor of Biochemistry and Molecular BiologyBone and cartilage pathogenesis of lysosomal storage disorders using zebrafish and cell culture systems, identification of novel congenital disorders of glycosylation, developmental role of the cation-independent mannose 6-phosphate receptor E-mail:
rsteet@ccrc.uga.edu
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Short Biography
Research Interests keywords and Selected Recent Publications All Publications |
Short Biography:Dr. Richard Steet received his B.A. in Biochemistry in 1994 from Colgate University and his Ph.D from the University of Colorado-Boulder in 2000. Prior to his Assistant Professor position at the University of Georgia, Dr. Steet was a Postdoctoral Fellow (2001-2005) and Instructor of Medicine (2005-2006) in the Department of Internal Medicine, Washington University School of Medicine. Full publications: 10To Top |
Research Interests:The importance of protein glycosylation in human development has been underscored by the emergence of a group of inherited disorders known as the congenital disorders of glycosylation (CDG). Using both cell biological and biochemical approaches, our laboratory seeks to identify and characterize novel forms of these disorders with particular interest in CDGs that arise from defects in proteins that support overall Golgi function (i.e. proteins involved in vesicular trafficking events). Utilizing zebrafish as a vertebrate model organism, we are also interested in studying the craniofacial pathogenesis of lysosomal storage diseases and related genetic disorders. The goal is to delineate the cellular and molecular events that lead to the developmental defects in these patients.To Top |
Keywords and Selected Recent Publications:Keywords: Congenital disorders of glycosylation, lysosomal storage disorders, zebrafish, development, Golgi function, vesicular trafficking
Recent Publications: Steet, R., S. Chung, B. Wustman, A. Powe, H. Do, and S. Kornfeld. 2006. The iminosugar isofagomine increases the activity of N3705 mutant acid-b-glucosidase in Gaucher fibroblasts by several mechanisms. Proc. Natl. Acad. Sci USA. 103: 13813-13818.
Steet, R. and S. Kornfeld. 2006. COG-7 deficient human fibroblasts exhibit altered recycling of Golgi proteins. Mol. Biol. Cell 17: 2312-2321.
Spaapen, L.J.M., J.A Bakker, S.B. van der Meer, H.J. Sijstermans, R. Steet, R.A. Wevers, and J. Jaeken. 2005. Clinical and biochemical presentation of siblings with COG-7 deficiency, a lethal multiple O- and N-glycosylation disorder. J. Inherit. Metab. Dis. 28: 707-714.
Steet, R., W.S. Lee, and S. Kornfeld. 2005. Identification of the minimal lysosomal enzyme recognition domain in cathepsin D. J. Biol. Chem. 280: 33318-33123.
Steet, R., R. Hullin, M. Kudo, M. Martinelli, N.U. Bosshard, T. Schaffner, S. Kornfeld, and B. Steinmann. 2005. A splicing mutation in a/b GlcNAc-1-phosphotransferase gene results in an adult onset form of mucolipidosis III associated with sensory neuropathy and cardiomyopathy. Am. J. Med. Genet. 132: 369-375.
Wu, X., R. Steet, O. Bohorov, J. Bakker, J. Newell, M. Krieger, L. Spaapen, S. Kornfeld, and H.H. Freeze. 2004. Mutation of the COG complex subunit gene COG7 causes a lethal congenital disorder. Nat. Med. 10: 518-523.
Steet, R., P. Melancon, and R.D. Kuchta. 2000. 3'-Azidothymidine potently inhibits the biosynthesis of highly branched N-linked oligosaccharides and poly-N-acetyllactosamine chains in cells. J. Biol. Chem. 275: 26812-26820.
Steet, R., M. Alizadeh, P. Melancon, and R.D. Kuchta. 1999. 3'-Azidothymidine significantly alters glycosphingolipid synthesis in melanoma cells and decreases the shedding of gangliosides. Glycoconj. J. 16: 237-245.
Yan, J.P., D.D. Ilsley, C. Frohlick, R. Steet, E.T. Hall, R.D. Kuchta, and P. Melancon. 1995. 3'Azidothymidine (zidovudine) inhibits glycosylation and dramatically alters glycosphingolipid synthesis in whole cells at clinically relevant concentrations. J. Biol. Chem. 270: 22836-22841. |
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