- ►asnjournals.org DP Germain, S Waldek, M Banikazemi, DA … - Journal of the American Society of Nephrology, 2007 - Am Soc Nephrol Fabry disease, an inherited deficiency of the lysosomal enzyme -galactosidase A,
causes progressive intralysosomal accumulation of globotriaosylceramide (GL-3)
and premature death from renal, cardiac, and cerebrovascular ... Cited by 95 - Related articles - All 6 versions
WR Wilcox, JP Oliveira, RJ Hopkin, A Ortiz, … - Molecular genetics and metabolism, 2007 - Elsevier Fabry disease (FD) is an X-linked lysosomal storage disease caused by
alpha-galactosidase A deficiency. The Fabry Registry is a global clinical effort
to collect longitudinal data on FD. In the past, most “carrier” females ... Cited by 61 - Related articles - All 4 versions
CM Eng, J Fletcher, WR Wilcox, S Waldek, CR … - Journal of inherited metabolic disease, 2007 - Springer J Inherit Metab Dis (2007) 30:184–192 DOI 10.1007/s10545-007-0521-2 ... Fabry
disease: Baseline medical characteristics of a cohort of 1765 ... CM Eng · J.
Fletcher · WR Wilcox · S. Waldek · CR Scott · DO Sillence · F. Breunig ... Cited by 58 - Related articles - BL Direct - All 5 versions
- ►angrylapdog.com R Schiffmann, H Askari, M Timmons, C … - Journal of the American Society of Nephrology, 2007 - jasn.org.p.angrylapdog.com This study was performed to determine whether adult male patients with Fabry
disease who demonstrate a continuing decline in renal function despite 2 to 4 yr
of conventionally dosed agalsidase alfa therapy (0.2 mg/kg every other week ... Cited by 43 - Related articles - All 8 versions
- ►asn-online.org [PDF] - Free from Publisher H Tahir, LL Jackson, DG Warnock - Journal of the American Society of Nephrology, 2007 - Am Soc Nephrol This report describes an open-label, nonrandomized, prospective evaluation of
the effects of angiotensin-converting enzyme inhibitor and angiotensin receptor
blocker therapy on patients who have Fabry disease and also received enzyme ... Cited by 35 - Related articles - All 8 versions
AC Vedder, GE Linthorst, G Houge, JEM … - PloS one, 2007 - pubmedcentral.nih.gov Conceived and designed the experiments: JA CH AV GL. Performed the experiments:
GH JG BB AH. Analyzed the data: CH AV. Contributed reagents/materials/analysis
tools: JG BB. Wrote the paper: CH AV GL GH. Other: Interpretation of data ... Cited by 31 - Related articles - All 9 versions
JE Wraith, A Tylki-Szymanska, N Guffon, YH … - The Journal of Pediatrics, 2008 - Elsevier Before treatment, results of skin biopsies from 12 male patients showed moderate
or severe GL-3 accumulation in superficial dermal capillary endothelial cells;
with treatment, these cells were completely cleared of GL-3 in week-24 ... Cited by 23 - Related articles - All 27 versions
T Ohashi, M Sakuma, T Kitagawa, K Suzuki, N … - Molecular Genetics and Metabolism, 2007 - Elsevier Fabry disease is a lysosomal storage disease characterized by deficient activity
of lysosomal α-galactosidase A (α-Gal A; EC 3.2.1.22). As a result,
glycolipids, particularly globotriaosylceramide (GL-3), progressively ... Cited by 22 - Related articles - All 2 versions
AC Vedder, F Breunig, WE Donker-Koopman, K … - Molecular genetics and metabolism, 2008 - Elsevier Two different enzyme preparations are used for the treatment of Fabry disease
patients, agalsidase alfa (Replagal, Shire) and agalsidase beta (Fabrazyme,
Genzyme). Therapeutic efficacy of both products has been variable probably ... Cited by 22 - Related articles - All 4 versions
