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Fabry disease in a large Nova Scotia kindred: carrier detection using leucocyte alpha-galactosidase activity and an NcoI polymorphism detected by an alpha-galactosidase cDNA clone.

Fabry disease is an X linked recessive disorder of glycosphingolipid metabolism resulting from a deficiency of the lysosomal hydrolase alpha-galactosidase (alpha-gal). Measurement of the enzyme activity, however, is not an accurate method for identification of female carriers among at risk relatives...

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Sonraí Bibleagrafaíochta
Main Authors: Kirkilionis, A J, Riddell, D C, Spence, M W, Fenwick, R G
Formáid: Artigo
Teanga:Inglês
Foilsithe: 1991
Ábhair:
Rochtain Ar Líne:https://ncbi.nlm.nih.gov/pmc/articles/PMC1016823/
https://ncbi.nlm.nih.gov/pubmed/1677424
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