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Results: 1 to 20 of 157

1.

Reduced glucosylceramide in the mouse model of Fabry disease: correction by successful enzyme replacement therapy.

Quinta R, Rodrigues D, Assunção M, Macedo MF, Azevedo O, Cunha D, Oliveira P, Sá Miranda MC.

Gene. 2014 Feb 15;536(1):97-104. doi: 10.1016/j.gene.2013.11.073. Epub 2013 Dec 13.

PMID:
24334116
[PubMed - indexed for MEDLINE]
2.

Plasma globotriaosylsphingosine as a biomarker of Fabry disease.

Togawa T, Kodama T, Suzuki T, Sugawara K, Tsukimura T, Ohashi T, Ishige N, Suzuki K, Kitagawa T, Sakuraba H.

Mol Genet Metab. 2010 Jul;100(3):257-61. doi: 10.1016/j.ymgme.2010.03.020. Epub 2010 Apr 1.

PMID:
20409739
[PubMed - indexed for MEDLINE]
3.

Enzyme replacement therapy partially prevents invariant Natural Killer T cell deficiency in the Fabry disease mouse model.

Macedo MF, Quinta R, Pereira CS, Sa Miranda MC.

Mol Genet Metab. 2012 May;106(1):83-91. doi: 10.1016/j.ymgme.2012.02.014. Epub 2012 Mar 1.

PMID:
22425450
[PubMed - indexed for MEDLINE]
4.

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.

Marshall J, Ashe KM, Bangari D, McEachern K, Chuang WL, Pacheco J, Copeland DP, Desnick RJ, Shayman JA, Scheule RK, Cheng SH.

PLoS One. 2010 Nov 24;5(11):e15033. doi: 10.1371/journal.pone.0015033.

PMID:
21124789
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.

Young-Gqamana B, Brignol N, Chang HH, Khanna R, Soska R, Fuller M, Sitaraman SA, Germain DP, Giugliani R, Hughes DA, Mehta A, Nicholls K, Boudes P, Lockhart DJ, Valenzano KJ, Benjamin ER.

PLoS One. 2013;8(3):e57631. doi: 10.1371/journal.pone.0057631. Epub 2013 Mar 5.

PMID:
23472096
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Treatment of neutral glycosphingolipid lysosomal storage diseases via inhibition of the ABC drug transporter, MDR1. Cyclosporin A can lower serum and liver globotriaosyl ceramide levels in the Fabry mouse model.

Mattocks M, Bagovich M, De Rosa M, Bond S, Binnington B, Rasaiah VI, Medin J, Lingwood C.

FEBS J. 2006 May;273(9):2064-75.

PMID:
16724420
[PubMed - indexed for MEDLINE]
7.

Reduction of elevated plasma globotriaosylsphingosine in patients with classic Fabry disease following enzyme replacement therapy.

van Breemen MJ, Rombach SM, Dekker N, Poorthuis BJ, Linthorst GE, Zwinderman AH, Breunig F, Wanner C, Aerts JM, Hollak CE.

Biochim Biophys Acta. 2011 Jan;1812(1):70-6. doi: 10.1016/j.bbadis.2010.09.007. Epub 2010 Sep 17.

PMID:
20851180
[PubMed - indexed for MEDLINE]
Free Article
8.

Tissue and plasma globotriaosylsphingosine could be a biomarker for assessing enzyme replacement therapy for Fabry disease.

Togawa T, Kawashima I, Kodama T, Tsukimura T, Suzuki T, Fukushige T, Kanekura T, Sakuraba H.

Biochem Biophys Res Commun. 2010 Sep 3;399(4):716-20. doi: 10.1016/j.bbrc.2010.08.006. Epub 2010 Aug 6.

PMID:
20692233
[PubMed - indexed for MEDLINE]
9.

Characterization of Fabry mice treated with recombinant adeno-associated virus 2/8-mediated gene transfer.

Choi JO, Lee MH, Park HY, Jung SC.

J Biomed Sci. 2010 Apr 16;17:26. doi: 10.1186/1423-0127-17-26.

PMID:
20398385
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.

Shiozuka C, Taguchi A, Matsuda J, Noguchi Y, Kunieda T, Uchio-Yamada K, Yoshioka H, Hamanaka R, Yano S, Yokoyama S, Mannen K, Kulkarni AB, Furukawa K, Ishii S.

J Biochem. 2011 Feb;149(2):161-70. doi: 10.1093/jb/mvq125. Epub 2010 Oct 19.

PMID:
20961863
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

Expression of genes and their responses to enzyme replacement therapy in a Fabry disease mouse model.

Park ES, Choi JO, Park JW, Lee MH, Park HY, Jung SC.

Int J Mol Med. 2009 Sep;24(3):401-7.

PMID:
19639234
[PubMed - indexed for MEDLINE]
12.

An in vitro model of Fabry disease.

Shu L, Murphy HS, Cooling L, Shayman JA.

J Am Soc Nephrol. 2005 Sep;16(9):2636-45. Epub 2005 Jul 20.

PMID:
16033856
[PubMed - indexed for MEDLINE]
Free Article
13.

Cardiomyopathy and response to enzyme replacement therapy in a male mouse model for Fabry disease.

Nguyen Dinh Cat A, Escoubet B, Agrapart V, Griol-Charhbili V, Schoeb T, Feng W, Jaimes E, Warnock DG, Jaisser F.

PLoS One. 2012;7(5):e33743. doi: 10.1371/journal.pone.0033743. Epub 2012 May 4.

PMID:
22574107
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Long-term effect of antibodies against infused alpha-galactosidase A in Fabry disease on plasma and urinary (lyso)Gb3 reduction and treatment outcome.

Rombach SM, Aerts JM, Poorthuis BJ, Groener JE, Donker-Koopman W, Hendriks E, Mirzaian M, Kuiper S, Wijburg FA, Hollak CE, Linthorst GE.

PLoS One. 2012;7(10):e47805. doi: 10.1371/journal.pone.0047805. Epub 2012 Oct 19.

PMID:
23094092
[PubMed - indexed for MEDLINE]
Free PMC Article
15.

Correction of enzymatic and lysosomal storage defects in Fabry mice by adenovirus-mediated gene transfer.

Ziegler RJ, Yew NS, Li C, Cherry M, Berthelette P, Romanczuk H, Ioannou YA, Zeidner KM, Desnick RJ, Cheng SH.

Hum Gene Ther. 1999 Jul 1;10(10):1667-82.

PMID:
10428212
[PubMed - indexed for MEDLINE]
16.

Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells.

Takenaka T, Murray GJ, Qin G, Quirk JM, Ohshima T, Qasba P, Clark K, Kulkarni AB, Brady RO, Medin JA.

Proc Natl Acad Sci U S A. 2000 Jun 20;97(13):7515-20.

PMID:
10840053
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice.

Ioannou YA, Zeidner KM, Gordon RE, Desnick RJ.

Am J Hum Genet. 2001 Jan;68(1):14-25. Epub 2000 Dec 13.

PMID:
11115376
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer.

Park J, Murray GJ, Limaye A, Quirk JM, Gelderman MP, Brady RO, Qasba P.

Proc Natl Acad Sci U S A. 2003 Mar 18;100(6):3450-4. Epub 2003 Mar 6.

PMID:
12624185
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice.

Jung SC, Han IP, Limaye A, Xu R, Gelderman MP, Zerfas P, Tirumalai K, Murray GJ, During MJ, Brady RO, Qasba P.

Proc Natl Acad Sci U S A. 2001 Feb 27;98(5):2676-81.

PMID:
11226298
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Caveolin-associated accumulation of globotriaosylceramide in the vascular endothelium of alpha-galactosidase A null mice.

Shu L, Shayman JA.

J Biol Chem. 2007 Jul 20;282(29):20960-7. Epub 2007 May 29.

PMID:
17535804
[PubMed - indexed for MEDLINE]
Free Article

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