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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Hlavní autoři: Maxwell, Megan, Bjorkman, Jonas, Nguyen, Tam, Sharp, Peter, Finnie, John, Paterson, Carol, Tonks, Ian, Paton, Barbara C., Kay, Graham F., Crane, Denis I.
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Microbiology 2003
Témata:
Mammalian Genetic Models with Minimal or Complex Phenotypes
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC166343/
https://ncbi.nlm.nih.gov/pubmed/12897163
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1128/MCB.23.16.5947-5957.2003
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