Beta-glucosidase 1 (GBA1) is a second bile acid β-glucosidase in addition to β-glucosidase 2 (GBA2). Study in β-glucosidase deficient mice and humans

Beta-glucosidase 1 (GBA1; lysosomal glucocerebrosidase) and β-glucosidase 2 (GBA2, non-lysosomal glucocerebrosidase) both have glucosylceramide as a main natural substrate. The enzyme-deficient conditions with glucosylceramide accumulation are Gaucher disease (GBA1–/– in humans), modelled by the Gba...

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Autores principales: Harzer, Klaus, Blech-Hermoni, Yotam, Goldin, Ehud, Felderhoff-Mueser, Ursula, Igney, Claudia, Sidransky, Ellen, Yildiz, Yildiz
Formato: Artigo
Lenguaje:Inglês
Publicado: 2012
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3529407/
https://ncbi.nlm.nih.gov/pubmed/22659419
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbrc.2012.05.117
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