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Reversion of Hepatobiliary Alterations By Bone Marrow Transplantation in a Murine Model of Erythropoietic Protoporphyria

✍ Scribed by Antonio Fontanellas; Frèdèric Mazurier; Marc Landry; Laurence Taine; Carine Morel; Monique Larou; Jean-Yves Daniel; Xavier Montagutelli; Rafael Enriquez de Salamanca; Hubert de Verneuil


Book ID
111721281
Publisher
John Wiley and Sons
Year
2000
Tongue
English
Weight
776 KB
Volume
32
Category
Article
ISSN
0270-9139

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## Abstract ## Background Fabry disease (FD) is a lysosomal storage disorders characterized by a deficiency of the lysosomal enzyme, α‐galactosidase A. This results in the accumulation of glycolipids, mainly globotriaosylceramide (GL‐3), in the lysosomes of various organs. Although bone marrow tra