Steet R - Search Results

1

Analysis of mucolipidosis II/III GNPTAB missense mutations identifies domains of UDP-GlcNAc:lysosomal enzyme GlcNAc-1-phosphotransferase involved in catalytic function and lysosomal enzyme recognition.

Qian Y, van Meel E, Flanagan-Steet H, Yox A, Steet R, Kornfeld S. Qian Y, et al. Among authors: steet r. J Biol Chem. 2015 Jan 30;290(5):3045-56. doi: 10.1074/jbc.M114.612507. Epub 2014 Dec 11. J Biol Chem. 2015. PMID: 25505245 Free PMC article.

2

Mutation of the COG complex subunit gene COG7 causes a lethal congenital disorder.

Wu X, Steet RA, Bohorov O, Bakker J, Newell J, Krieger M, Spaapen L, Kornfeld S, Freeze HH. Wu X, et al. Nat Med. 2004 May;10(5):518-23. doi: 10.1038/nm1041. Epub 2004 Apr 25. Nat Med. 2004. PMID: 15107842

3

A splicing mutation in the alpha/beta GlcNAc-1-phosphotransferase gene results in an adult onset form of mucolipidosis III associated with sensory neuropathy and cardiomyopathy.

Steet RA, Hullin R, Kudo M, Martinelli M, Bosshard NU, Schaffner T, Kornfeld S, Steinmann B. Steet RA, et al. Am J Med Genet A. 2005 Feb 1;132A(4):369-75. doi: 10.1002/ajmg.a.30498. Am J Med Genet A. 2005. PMID: 15633164

4

Identification of the minimal lysosomal enzyme recognition domain in cathepsin D.

Steet R, Lee WS, Kornfeld S. Steet R, et al. J Biol Chem. 2005 Sep 30;280(39):33318-23. doi: 10.1074/jbc.M505994200. Epub 2005 Aug 4. J Biol Chem. 2005. PMID: 16081416 Free article.

5

COG-7-deficient Human Fibroblasts Exhibit Altered Recycling of Golgi Proteins.

Steet R, Kornfeld S. Steet R, et al. Mol Biol Cell. 2006 May;17(5):2312-21. doi: 10.1091/mbc.e05-08-0822. Epub 2006 Mar 1. Mol Biol Cell. 2006. PMID: 16510524 Free PMC article.

6

The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.

Steet RA, Chung S, Wustman B, Powe A, Do H, Kornfeld SA. Steet RA, et al. Proc Natl Acad Sci U S A. 2006 Sep 12;103(37):13813-8. doi: 10.1073/pnas.0605928103. Epub 2006 Aug 31. Proc Natl Acad Sci U S A. 2006. PMID: 16945909 Free PMC article.

7

Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase.

Steet R, Chung S, Lee WS, Pine CW, Do H, Kornfeld S. Steet R, et al. Biochem Pharmacol. 2007 May 1;73(9):1376-83. doi: 10.1016/j.bcp.2006.12.015. Epub 2006 Dec 15. Biochem Pharmacol. 2007. PMID: 17217920 Free PMC article.

8

Altered chondrocyte differentiation and extracellular matrix homeostasis in a zebrafish model for mucolipidosis II.

Flanagan-Steet H, Sias C, Steet R. Flanagan-Steet H, et al. Among authors: steet r. Am J Pathol. 2009 Nov;175(5):2063-75. doi: 10.2353/ajpath.2009.090210. Epub 2009 Oct 15. Am J Pathol. 2009. PMID: 19834066 Free PMC article.

9

Selective yolk deposition and mannose phosphorylation of lysosomal glycosidases in zebrafish.

Fan X, Klein M, Flanagan-Steet HR, Steet R. Fan X, et al. Among authors: steet r. J Biol Chem. 2010 Oct 22;285(43):32946-32953. doi: 10.1074/jbc.M110.158295. Epub 2010 Aug 20. J Biol Chem. 2010. PMID: 20729204 Free PMC article.

10

Extensive mannose phosphorylation on leukemia inhibitory factor (LIF) controls its extracellular levels by multiple mechanisms.

Barnes J, Lim JM, Godard A, Blanchard F, Wells L, Steet R. Barnes J, et al. Among authors: steet r. J Biol Chem. 2011 Jul 15;286(28):24855-64. doi: 10.1074/jbc.M111.221432. Epub 2011 May 25. J Biol Chem. 2011. PMID: 21613225 Free PMC article.

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