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- Title
To see a world in a grain of sand: elucidating the pathophysiology of Anderson–Fabry disease through investigations of a cellular model.
- Authors
Pastores, Gregory M.; Hughes, Derralynn A.
- Abstract
Thomaidis and colleagues have created a cellular model of Anderson–Fabry disease by ‘silencing’ α-galactosidase A (AGAL) activity in human tubular epithelial cells. Increased membrane globotriaosylceramide (Gb3/CD77) expression was observed; it is suggested that this finding may be potentially useful as a surrogate marker of disease severity. Decreased membrane Gb3/CD77 expression was observed following agalsidase-α treatment, providing evidence of changes in cellular phenotype in response to enzyme therapy.Kidney International (2009) 75, 351–353; doi:10.1038/ki.2008.606
- Subjects
KIDNEY diseases; PATHOLOGICAL physiology; EPITHELIAL cells; CELL membranes; NEPHROLOGY
- Publication
Kidney International, 2009, Vol 75, Issue 4, p351
- ISSN
0085-2538
- Publication type
Article
- DOI
10.1038/ki.2008.606