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Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder

BACKGROUND: Zellweger spectrum disorders (ZSDs) are multisystem genetic disorders caused by a lack of functional peroxisomes, due to mutations in one of the PEX genes, encoding proteins involved in peroxisome biogenesis. The phenotypic spectrum of ZSDs ranges from an early lethal form to much milder...

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Detalles Bibliográficos
Autores principales: Berendse, Kevin, Ebberink, Merel S, IJlst, Lodewijk, Poll-The, Bwee Tien, Wanders, Ronald J A, Waterham, Hans R
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3844471/
https://www.ncbi.nlm.nih.gov/pubmed/24016303
http://dx.doi.org/10.1186/1750-1172-8-138