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Items: 1 to 20 of 88

1.

Global gene expression profile progression in Gaucher disease mouse models.

Xu YH, Jia L, Quinn B, Zamzow M, Stringer K, Aronow B, Sun Y, Zhang W, Setchell KD, Grabowski GA.

BMC Genomics. 2011 Jan 11;12:20. doi: 10.1186/1471-2164-12-20.

2.

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.

Hum Mol Genet. 2010 Mar 15;19(6):1088-97. doi: 10.1093/hmg/ddp580. Epub 2010 Jan 4.

3.

Gaucher disease: transcriptome analyses using microarray or mRNA sequencing in a Gba1 mutant mouse model treated with velaglucerase alfa or imiglucerase.

Dasgupta N, Xu YH, Oh S, Sun Y, Jia L, Keddache M, Grabowski GA.

PLoS One. 2013 Oct 4;8(10):e74912. doi: 10.1371/journal.pone.0074912. eCollection 2013.

4.

Viable mouse models of acid beta-glucosidase deficiency: the defect in Gaucher disease.

Xu YH, Quinn B, Witte D, Grabowski GA.

Am J Pathol. 2003 Nov;163(5):2093-101.

5.

Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.

Barnes S, Xu YH, Zhang W, Liou B, Setchell KD, Bao L, Grabowski GA, Sun Y.

PLoS One. 2014 Dec 31;9(12):e116023. doi: 10.1371/journal.pone.0116023. eCollection 2014.

6.

Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease.

Sun Y, Zhang W, Xu YH, Quinn B, Dasgupta N, Liou B, Setchell KD, Grabowski GA.

PLoS One. 2013;8(3):e57560. doi: 10.1371/journal.pone.0057560. Epub 2013 Mar 8.

7.

Down-regulation of Bcl-2 in the fetal brain of the Gaucher disease mouse model: a possible role in the neuronal loss.

Hong YB, Kim EY, Jung SC.

J Hum Genet. 2004;49(7):349-54. Epub 2004 Jun 4.

PMID:
15179559
8.

Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration.

Farfel-Becker T, Vitner EB, Kelly SL, Bame JR, Duan J, Shinder V, Merrill AH Jr, Dobrenis K, Futerman AH.

Hum Mol Genet. 2014 Feb 15;23(4):843-54. doi: 10.1093/hmg/ddt468. Epub 2013 Sep 24.

9.

Induction of the type I interferon response in neurological forms of Gaucher disease.

Vitner EB, Farfel-Becker T, Ferreira NS, Leshkowitz D, Sharma P, Lang KS, Futerman AH.

J Neuroinflammation. 2016 May 12;13(1):104. doi: 10.1186/s12974-016-0570-2.

10.

AAV8-mediated expression of glucocerebrosidase ameliorates the storage pathology in the visceral organs of a mouse model of Gaucher disease.

McEachern KA, Nietupski JB, Chuang WL, Armentano D, Johnson J, Hutto E, Grabowski GA, Cheng SH, Marshall J.

J Gene Med. 2006 Jun;8(6):719-29.

PMID:
16528760
11.
12.

Gaucher disease: understanding the molecular pathogenesis of sphingolipidoses.

Cox TM.

J Inherit Metab Dis. 2001;24 Suppl 2:106-21; discussion 87-8. Review.

PMID:
11758671
13.

Global gene expression in a type 2 Gaucher disease brain.

Myerowitz R, Mizukami H, Richardson KL, Finn LS, Tifft CJ, Proia RL.

Mol Genet Metab. 2004 Dec;83(4):288-96.

PMID:
15589115
14.

Neuronal forms of Gaucher disease.

Vitner EB, Futerman AH.

Handb Exp Pharmacol. 2013;(216):405-19. doi: 10.1007/978-3-7091-1511-4_20. Review.

PMID:
23563668
15.

Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model.

Dasgupta N, Xu YH, Li R, Peng Y, Pandey MK, Tinch SL, Liou B, Inskeep V, Zhang W, Setchell KD, Keddache M, Grabowski GA, Sun Y.

Hum Mol Genet. 2015 Dec 15;24(24):7031-48. doi: 10.1093/hmg/ddv404. Epub 2015 Sep 29.

16.

Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.

Mizukami H, Mi Y, Wada R, Kono M, Yamashita T, Liu Y, Werth N, Sandhoff R, Sandhoff K, Proia RL.

J Clin Invest. 2002 May;109(9):1215-21.

17.

Generation of a conditional knockout of murine glucocerebrosidase: utility for the study of Gaucher disease.

Sinclair GB, Jevon G, Colobong KE, Randall DR, Choy FY, Clarke LA.

Mol Genet Metab. 2007 Feb;90(2):148-56. Epub 2006 Oct 31.

PMID:
17079175
18.

Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide.

Bodennec J, Pelled D, Riebeling C, Trajkovic S, Futerman AH.

FASEB J. 2002 Nov;16(13):1814-6. Epub 2002 Sep 5.

PMID:
12223447
19.

Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice.

Sun Y, Jia L, Williams MT, Zamzow M, Ran H, Quinn B, Aronow BJ, Vorhees CV, Witte DP, Grabowski GA.

BMC Neurosci. 2008 Aug 1;9:76. doi: 10.1186/1471-2202-9-76.

20.

Peripheral arterial occlusive disease: global gene expression analyses suggest a major role for immune and inflammatory responses.

Fu S, Zhao H, Shi J, Abzhanov A, Crawford K, Ohno-Machado L, Zhou J, Du Y, Kuo WP, Zhang J, Jiang M, Jin JG.

BMC Genomics. 2008 Aug 1;9:369. doi: 10.1186/1471-2164-9-369.

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