Pexophagy is responsible for 65% of cases of peroxisome biogenesis disorders
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چکیده
منابع مشابه
Pexophagy is responsible for 65% of cases of peroxisome biogenesis disorders
Peroxisome biogenesis disorders (PBDs) is a group of diseases caused by mutations in one of the peroxins, proteins responsible for biogenesis of the peroxisomes. In recent years, it became clear that many peroxins (e.g., PEX3 and PEX14) play additional roles in peroxisome homeostasis (such as promoting autophagic degradation of peroxisomes or pexophagy), which are often opposite to their origin...
متن کاملThe peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders
Peroxisome biogenesis disorders (PBDs) are metabolic disorders caused by the loss of peroxisomes. The majority of PBDs result from mutation in one of 3 genes that encode for the peroxisomal AAA ATPase complex (AAA-complex) required for cycling PEX5 for peroxisomal matrix protein import. Mutations in these genes are thought to result in a defect in peroxisome assembly by preventing the import of...
متن کاملPeroxisome biogenesis disorders
Defects in PEX genes impair peroxisome assembly and multiple metabolic pathways confined to this organelle, thus providing the biochemical and molecular bases of the peroxisome biogenesis disorders (PBD). PBD are divided into two types--Zellweger syndrome spectrum (ZSS) and rhizomelic chondrodysplasia punctata (RCDP). Biochemical studies performed in blood and urine are used to screen for the P...
متن کاملdiagnosis of human peroxisomal biogenesis disorders: molecular understanding of peroxisome
peroxisomes are single membrane bound organelles present in a wide variety of eukaryotes from yeast to human, have different functions, two of which are well conserved, i.e. hydrogen peroxide decomposition and fatty acid beta-oxidation. the process of peroxisome biogenesis can be divided into distinct steps including peroxisome membrane assembly, import of matrix proteins and peroxisome prolife...
متن کاملPeroxisome biogenesis and human peroxisome-deficiency disorders
Peroxisome is a single-membrane-bounded ubiquitous organelle containing a hundred different enzymes that catalyze various metabolic pathways such as β-oxidation of very long-chain fatty acids and synthesis of plasmalogens. To investigate peroxisome biogenesis and human peroxisome biogenesis disorders (PBDs) including Zellweger syndrome, more than a dozen different complementation groups of Chin...
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ژورنال
عنوان ژورنال: Autophagy
سال: 2017
ISSN: 1554-8627,1554-8635
DOI: 10.1080/15548627.2017.1291480