Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities

INTRODUCTION: Zellweger spectrum disorder (PBD-ZSD) is a disease continuum caused by mutations in a subset of PEX genes required for normal peroxisome assembly and function. They highlight the importance of peroxisomes in the development and functions of the central nervous system, liver, and other...

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Publicado en:Stem Cell Res Ther
Autores principales: Wang, Xiao-Ming, Yik, Wing Yan, Zhang, Peilin, Lu, Wange, Huang, Ning, Kim, Bo Ram, Shibata, Darryl, Zitting, Madison, Chow, Robert H., Moser, Ann B., Steinberg, Steven J., Hacia, Joseph G.
Formato: Artigo
Lenguaje:Inglês
Publicado: BioMed Central 2015
Materias:
Research
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC4553005/
https://ncbi.nlm.nih.gov/pubmed/26319495
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13287-015-0149-3
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