Mahuran DJ - Search Results

1

Lysosomal abnormalities in hereditary spastic paraplegia types SPG15 and SPG11.

Renvoisé B, Chang J, Singh R, Yonekawa S, FitzGibbon EJ, Mankodi A, Vanderver A, Schindler A, Toro C, Gahl WA, Mahuran DJ, Blackstone C, Pierson TM. Renvoisé B, et al. Among authors: mahuran dj. Ann Clin Transl Neurol. 2014 Jun 1;1(6):379-389. doi: 10.1002/acn3.64. Ann Clin Transl Neurol. 2014. PMID: 24999486 Free PMC article.

2

A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysates.

Tropak MB, Bukovac SW, Rigat BA, Yonekawa S, Wakarchuk W, Mahuran DJ. Tropak MB, et al. Among authors: mahuran dj. Glycobiology. 2010 Mar;20(3):356-65. doi: 10.1093/glycob/cwp183. Epub 2009 Nov 16. Glycobiology. 2010. PMID: 19917668 Free PMC article.

3

Juvenile-onset motor neuron disease caused by novel mutations in β-hexosaminidase.

Pierson TM, Torres PA, Zeng BJ, Glanzman AM, Adams D, Finkel RS, Mahuran DJ, Pastores GM, Tennekoon GI, Kolodny EH. Pierson TM, et al. Among authors: mahuran dj. Mol Genet Metab. 2013 Jan;108(1):65-9. doi: 10.1016/j.ymgme.2012.10.023. Epub 2012 Nov 2. Mol Genet Metab. 2013. PMID: 23158871 Free PMC article.

4

Rapid assembly of a library of lipophilic iminosugars via the thiol-ene reaction yields promising pharmacological chaperones for the treatment of Gaucher disease.

Goddard-Borger ED, Tropak MB, Yonekawa S, Tysoe C, Mahuran DJ, Withers SG. Goddard-Borger ED, et al. Among authors: mahuran dj. J Med Chem. 2012 Mar 22;55(6):2737-45. doi: 10.1021/jm201633y. Epub 2012 Mar 6. J Med Chem. 2012. PMID: 22360565 Free PMC article.

5

Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo.

Tropak MB, Yonekawa S, Karumuthil-Melethil S, Thompson P, Wakarchuk W, Gray SJ, Walia JS, Mark BL, Mahuran D. Tropak MB, et al. Mol Ther Methods Clin Dev. 2016 Mar 2;3:15057. doi: 10.1038/mtm.2015.57. eCollection 2016. Mol Ther Methods Clin Dev. 2016. PMID: 26966698 Free PMC article.

6

Pyrimethamine Derivatives: Insight into Binding Mechanism and Improved Enhancement of Mutant β-N-acetylhexosaminidase Activity.

Tropak MB, Zhang J, Yonekawa S, Rigat BA, Aulakh VS, Smith MR, Hwang HJ, Ciufolini MA, Mahuran DJ. Tropak MB, et al. Among authors: mahuran dj. J Med Chem. 2015 Jun 11;58(11):4483-93. doi: 10.1021/jm5017895. Epub 2015 Jun 2. J Med Chem. 2015. PMID: 25984755

7

In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside.

Sinici I, Yonekawa S, Tkachyova I, Gray SJ, Samulski RJ, Wakarchuk W, Mark BL, Mahuran DJ. Sinici I, et al. Among authors: mahuran dj. PLoS One. 2013;8(3):e57908. doi: 10.1371/journal.pone.0057908. Epub 2013 Mar 4. PLoS One. 2013. PMID: 23483939 Free PMC article.

8

Synthesis of 1,5-dideoxy-1,5-iminoribitol C-glycosides through a nitrone-olefin cycloaddition domino strategy: identification of pharmacological chaperones of mutant human lysosomal β-galactosidase.

Siriwardena A, Sonawane DP, Bande OP, Markad PR, Yonekawa S, Tropak MB, Ghosh S, Chopade BA, Mahuran DJ, Dhavale DD. Siriwardena A, et al. Among authors: mahuran dj. J Org Chem. 2014 May 16;79(10):4398-404. doi: 10.1021/jo500328u. Epub 2014 Apr 28. J Org Chem. 2014. PMID: 24735108

9

Presenilin-1, nicastrin, amyloid precursor protein, and gamma-secretase activity are co-localized in the lysosomal membrane.

Pasternak SH, Bagshaw RD, Guiral M, Zhang S, Ackerley CA, Pak BJ, Callahan JW, Mahuran DJ. Pasternak SH, et al. Among authors: mahuran dj. J Biol Chem. 2003 Jul 18;278(29):26687-94. doi: 10.1074/jbc.m304009200. Epub 2003 May 7. J Biol Chem. 2003. PMID: 12736250 Free article.

10

Lysosomal membrane proteomics and biogenesis of lysosomes.

Bagshaw RD, Mahuran DJ, Callahan JW. Bagshaw RD, et al. Among authors: mahuran dj. Mol Neurobiol. 2005 Aug;32(1):27-41. doi: 10.1385/MN:32:1:027. Mol Neurobiol. 2005. PMID: 16077181 Review.

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