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Pex13 Inactivation in the Mouse Disrupts Peroxisome Biogenesis and Leads to a Zellweger Syndrome Phenotype

Zellweger syndrome is the archetypical peroxisome biogenesis disorder and is characterized by defective import of proteins into the peroxisome, leading to peroxisomal metabolic dysfunction and widespread tissue pathology. In humans, mutations in the PEX13 gene, which encodes a peroxisomal membrane p...

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Publicado no:Mol Cell Biol
Principais autores: Maxwell, Megan, Bjorkman, Jonas, Nguyen, Tam, Sharp, Peter, Finnie, John, Paterson, Carol, Tonks, Ian, Paton, Barbara C., Kay, Graham F., Crane, Denis I.
Formato: Artigo
Idioma:Inglês
Publicado em: Taylor & Francis 2003
Assuntos:
Mammalian Genetic Models with Minimal or Complex Phenotypes
Acesso em linha:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC166343/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/12897163/
https://ncbi.nlm.nih.govhttps://doi.org/10.1128/MCB.23.16.5947-5957.2003
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