Grabowski GA - Search Results

1

Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome.

Campeau PM, Rafei M, Boivin MN, Sun Y, Grabowski GA, Galipeau J. Campeau PM, et al. Among authors: grabowski ga. Blood. 2009 Oct 8;114(15):3181-90. doi: 10.1182/blood-2009-02-205708. Epub 2009 Jul 8. Blood. 2009. PMID: 19587377 Free PMC article.

2

Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation.

Sun Y, Qi X, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. J Biol Chem. 2003 Aug 22;278(34):31918-23. doi: 10.1074/jbc.M302752200. Epub 2003 Jun 17. J Biol Chem. 2003. PMID: 12813057 Free article.

3

Gaucher disease mouse models: point mutations at the acid beta-glucosidase locus combined with low-level prosaposin expression lead to disease variants.

Sun Y, Quinn B, Witte DP, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. J Lipid Res. 2005 Oct;46(10):2102-13. doi: 10.1194/jlr.M500202-JLR200. Epub 2005 Aug 1. J Lipid Res. 2005. PMID: 16061944 Free article.

4

Conditional expression of human acid beta-glucosidase improves the visceral phenotype in a Gaucher disease mouse model.

Sun Y, Quinn B, Xu YH, Leonova T, Witte DP, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. J Lipid Res. 2006 Oct;47(10):2161-70. doi: 10.1194/jlr.M600239-JLR200. Epub 2006 Jul 21. J Lipid Res. 2006. PMID: 16861620 Free article.

5

Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking.

Sun Y, Witte DP, Zamzow M, Ran H, Quinn B, Matsuda J, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. Hum Mol Genet. 2007 Apr 15;16(8):957-71. doi: 10.1093/hmg/ddm040. Epub 2007 Mar 12. Hum Mol Genet. 2007. PMID: 17353235

6

Involvement of acid beta-glucosidase 1 in the salvage pathway of ceramide formation.

Kitatani K, Sheldon K, Rajagopalan V, Anelli V, Jenkins RW, Sun Y, Grabowski GA, Obeid LM, Hannun YA. Kitatani K, et al. Among authors: grabowski ga. J Biol Chem. 2009 May 8;284(19):12972-8. doi: 10.1074/jbc.M802790200. Epub 2009 Mar 11. J Biol Chem. 2009. PMID: 19279011 Free PMC article.

7

In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models.

Sun Y, Liou B, Quinn B, Ran H, Xu YH, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. PLoS One. 2009 Oct 7;4(10):e7320. doi: 10.1371/journal.pone.0007320. PLoS One. 2009. PMID: 19809509 Free PMC article.

8

Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits.

Sun Y, Liou B, Ran H, Skelton MR, Williams MT, Vorhees CV, Kitatani K, Hannun YA, Witte DP, Xu YH, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. Hum Mol Genet. 2010 Mar 15;19(6):1088-97. doi: 10.1093/hmg/ddp580. Epub 2010 Jan 4. Hum Mol Genet. 2010. PMID: 20047948 Free PMC article.

9

Multi-system disorders of glycosphingolipid and ganglioside metabolism.

Xu YH, Barnes S, Sun Y, Grabowski GA. Xu YH, et al. Among authors: grabowski ga. J Lipid Res. 2010 Jul;51(7):1643-75. doi: 10.1194/jlr.R003996. Epub 2010 Mar 8. J Lipid Res. 2010. PMID: 20211931 Free PMC article. Review.

10

Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease.

Sun Y, Grabowski GA. Sun Y, et al. Among authors: grabowski ga. Autophagy. 2010 Jul;6(5):648-9. doi: 10.4161/auto.6.5.12047. Epub 2010 Jul 1. Autophagy. 2010. PMID: 20458183

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