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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Autors principals: Berendse, Kevin, Ebberink, Merel S, IJlst, Lodewijk, Poll-The, Bwee Tien, Wanders, Ronald J A, Waterham, Hans R
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2013
Matèries:
Research
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3844471/
https://ncbi.nlm.nih.gov/pubmed/24016303
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1750-1172-8-138
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