PEX11β Deficiency Is Lethal and Impairs Neuronal Migration but Does Not Abrogate Peroxisome Function

Zellweger syndrome is a lethal neurological disorder characterized by severe defects in peroxisomal protein import. The resulting defects in peroxisome metabolism and the accumulation of peroxisomal substrates are thought to cause the other Zellweger syndrome phenotypes, including neuronal migration...

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Autori principali: Li, Xiaoling, Baumgart, Eveline, Morrell, James C., Jimenez-Sanchez, Gerardo, Valle, David, Gould, Stephen J.
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society for Microbiology 2002
Soggetti:
Mammalian Genetic Models with Minimal or Complex Phenotypes
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC133847/
https://ncbi.nlm.nih.gov/pubmed/12024045
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1128/MCB.22.12.4358-4365.2002
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