Enhanced thrombospondin-1 causes dysfunction of vascular endothelial cells derived from Fabry disease-induced pluripotent stem cells

BACKGROUND: Fabry disease (FD) is a recessive X-linked lysosomal storage disorder caused by α-galactosidase A (GLA) deficiency. Although the mechanism is unclear, GLA deficiency causes an accumulation of globotriaosylceramide (Gb3), leading to vasculopathy. METHODS: To explore the relationship betwe...

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Detalles Bibliográficos
Autores principales: Do, Hyo-Sang, Park, Sang-Wook, Im, Ilkyun, Seo, Donghyuk, Yoo, Han-Wook, Go, Heounjeong, Kim, Yoo Hyung, Koh, Gou Young, Lee, Beom-Hee, Han, Yong-Mahn
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Research paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6992938/
https://www.ncbi.nlm.nih.gov/pubmed/31981984
http://dx.doi.org/10.1016/j.ebiom.2020.102633

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