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Richard Steet
Associate Professor of Biochemistry and Molecular Biology


Bone 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
Telephone: 706-583-5550
Fax: 706-542-4412

Short Biography
Research Interests
Publications

BCMB 8160

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: 10

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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.

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Publications: Author's Last Name: Steet

Journal Articles
Book Chapters are listed at the bottom of this page.

L Boccuto, K Aoki, H Flanagan-Steet, CF Chen, X Fan, F Bartel, M Petukh, A Pittman, R Saul, A Chaubey, E Alexov, M Tiemeyer, R Steet, CE Schwartz. 2014. A mutation in a ganglioside biosynthetic enzyme, ST3GAL5, results in salt & pepper syndrome, a neurocutaneous disorder with altered glycolipid and glycoprotein glycosylation . Hum Mol Genet 23(2): 418-433. PMID:24026681

JG Leroy, D Sillence, T Wood, J Barnes, RR Lebel, MJ Friez, RE Stevenson, R Steet, SS Cathey. 2014. A novel intermediate mucolipidosis II/III alpha beta caused by GNPTAB mutation in the cytosolic N-terminal domain.. Eur J Hum Genet 5: 594-601. PMID:24045841

N.E. Mbua, H.R. Flanagan-Steet, S. Johnson, M.A. Wolfert, G.J. Boons, R. Steet. 2013. Abnormal accumulation and recycling of glycoproteins visualized in Niemann-Pick type C cells using the chemical reporter strategy. Proc. Natl. Acad. Sci USA 110: 10207-10212. PMID:23733943

N.E. Mbua, X. Li, H.R. Flanagan-Steet, L. Meng, K. Aoki, K.W. Moremen, M.A. Wolfert, R. Steet, G.J. Boons. 2013. Selective exo-enzymatic labeling (SEEL) of N-glycans of living cells by recombinant ST6Gal. . Angewandte 52: 13012-13015. PMID:24129959

JG Leroy, D Sillence, T Wood, J Barnes, RR Lebel, MJ Friez, RE Stevenson, R Steet, SS Cathey. 2013. A novel intermediate mucolipidosis II/IIIalpha/beta caused by GNPTAB mutation in the cytosolic N-terminal domain. Eur J Hum Genet 10.1038: -. PMID:24045841

Y Qian, H Flanagan-Steet, E van Meel, R Steet, SA Kornfeld, . 2013. The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphototransferase is a substrate recognition module. Proc Natl Acad Sci USA 110(25): 10246-10251. PMID:23733939

J Chu, A Mir, N Gao, S Rosa, C Monson, V Sharma, R Steet, HH Freeze, MA Lehrman, KC Sadler. 2013. A zebrafish model of congenital disorders of glycosylation with phosphomannose isomerase deficiency reveals an early opportunity for corrective mannose supplementation. Dis Model Mech 6(1): 95-105. PMID:22899857

HR Flanagan-Steet, R Steet. 2013. "Casting" light on the role of glycosylation during embryonic development: insights from zebrafish. Glycoconj J 30 (1): 33-40. PMID:22638861

F. Friscourt, P.A. Ledin, N.E. Mbua, H.R. Flanagan-Steet, M.A. Wolfert, R. Steet, G.-J. Boons. 2012. Polar dibenzocyclooctynes for selective labeling of extracellular glycoconjugates of living cells. J. Am. Chem. Soc. 134: 5381-5389. PMID:22376061

J. Barnes, D. Warejcka, J. Simpliciano, S. Twining, R. Steet. 2012. The latency-associated peptide of transforming growth factor beta is not subject to physiological mannose phosphorylation. J. Biol. Chem. 287: 7526-7534. PMID:22262853

A.C. Petrey, H. Flanagan-Steet, S. Johnson, X. Fan, M. Dela Rosa, M.E. Haskins, A.V. Nairn, K.W. Moremen, R. Steet. 2012. Excessive activity of cathepsin K is associated with cartilage defects in a zebrafish model of mucolipidosis II. . Dis Model Mech 5: 177-190. PMID:22046029

H. Flanagan-Steet, R. Steet. 2012. "Casting" light on the role of glycosylation during embryonic development: Insights from zebrafish. Glycoconj. J. In press.

M.J. Passineau, L. Machen, L. Zourelias, K. Nega, R. Paul, M.J. Macdougall, O. Mamaeva, R.L. Benza, R. Steet, J. Barnes, H.M. Kingston, T. Fahrenholz. 2011. α-Galactosidase A expressed in the salivary glands partially corrects organ biochemical deficits in the Fabry mouse through endocrine trafficking. Hum. Gene Ther. 22: 293-301. PMID:20858137

N.E. Mbua, J. Guo, M.A. Wolfert, R. Steet, G.J. Boons. 2011. Strain-promoted alkyne-azide cycloadditions (SPAAC) reveal new features of glycoconjugate biosynthesis. ChemBioChem 12: 1912-1921.

J. Barnes, J.M Lim, A. Godard, F. Blanchard, L. Wells, R. Steet. 2011. Extensive mannose phosphorylation on leugemic inhibitory factor (LIF)controls its extracellular levels by multiple mechanisms. J. Biol. Chem. 286: 24855-24864. PMID:21613225

S. Baas, M. Sharrow, V. Kotu, M. Middleton, K. Nguyen, H. Flanagan-Steet, K. Aoki, M. Tiemeyer. 2011. Sugar-free frosting, a homolog of SAD kinase, drives neural-specific glycan expression in the Drosophila embryo. Development 138: 553-563. PMID:21205799

X. Fan, M. Klein, H.R. Flanagan-Steet, R. Steet. 2010. Selective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafish. J. Biol. Chem. 285: 32946-32953. PMID:20729204

H. Flanagan-Steet, C. Sias, R. Ateet. 2009. Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II. Am. J. Pathol. 175: 2063-2075. PMID:19834066

R. Steet, S. Chung, W.S. Lee, C.W. Pine, H. Do, S. Kornfeld. 2007. Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase . Biochem. Pharmacol. 73: 1376-1383. PMID:17217920

R. Steet, S. Kornfeld. 2006. COG-7 deficient human fibroblasts exhibit altered recycling of Golgi proteins. Mol. Biol. Cell 17: 2312-2321. PMID:16510524

R. Steet, S. Chung, B. Wustman, D.A. Powell, S.H. Doares, S. Kornfeld. 2006. The iminosugar isofagomine increases the activity of N370S mutant acid-beta-glucosidase in Gaucher fibroblasts by several mechanisms. Proc. Natl. Acad. Sci. USA 103: 13813-13818. PMID:16945909

Book Chapters

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