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The PEX1/PEX6 AAA-ATPase is required for the biogenesis and maintenance of peroxisomes. Mutations in HsPEX1 and HsPEX6 disrupt peroxisomal matrix protein import and are the leading cause of Peroxisome Biogenesis Disorders (PBDs). The most common disease-causing mutation in PEX1 is the HsPEX1G843D al...
ORGANISM(S): Homo sapiens (Human) 
2025-04-28 | PXD061295 | Pride
Identification of co-purified interaction partners of Pex1/Pex6 complex
ORGANISM(S): Saccharomyces cerevisiae (Baker's yeast) 
2023-10-24 | PXD043907 | Pride
Human peroxisome biogenesis disorders are lethal genetic disease in which abnormal peroxisome assembly compromises overall peroxisome and cellular function. Peroxisomes are ubiquitous membrane-bound organelles involved in several important biochemical processes, notably lipid metabolism and the use ...
ORGANISM(S): Drosophila melanogaster 
Gene expression analysis of retinas from a mouse model of the mild form of Zellweger spectrum disorder (ZSD). Mice homozygous for the hypomorphic Pex1-G844D allele, the murine ortholog of the human PEX1-G843D mutation found in a subset of patients with autosomal recessive ZSD, develop phenotypes fou...
ORGANISM(S): Mus musculus 
2013-12-15 | GSE52348 | GEO
Gene expression analysis of retinas from a mouse model of the mild form of Zellweger spectrum disorder (ZSD). Mice homozygous for the hypomorphic Pex1-G844D allele, the murine ortholog of the human PEX1-G843D mutation found in a subset of patients with autosomal recessive ZSD, develop phenotypes f...
ORGANISM(S): Mus musculus 
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