The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders

@inproceedings{Law2017ThePA,
  title={The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders},
  author={Kelsey B. Law and Dana Bronte-Tinkew and Erminia Di Pietro and Ann Snowden and Richard P. O. Jones and Ann Moser and John H Brumell and Nancy E. Braverman and Peter Kijun Kim},
  booktitle={Autophagy},
  year={2017}
}
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 matrix proteins. However, we show here that loss of the AAA-complex does not prevent matrix protein… CONTINUE READING
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A pex1 missense mutation improves peroxisome function in a subset of Arabidopsis pex6 mutants without restoring PEX5 recycling.

Proceedings of the National Academy of Sciences of the United States of America • 2018
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