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Literature summary for 3.6.4.7 extracted from

  • Law, K.B.; Bronte-Tinkew, D.; Di Pietro, E.; Snowden, A.; Jones, R.O.; Moser, A.; Brumell, J.H.; Braverman, N.; Kim, P.K.
    The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders (2017), Autophagy, 13, 868-884 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.6.4.7

Protein Variants

Protein Variants Comment Organism
G843D the mutation of the PEX1 component causes a mild form of peroxisome biogenesis disorder Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
peroxisome
-
 Homo sapiens 5777
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O Homo sapiens
-
 ADP + phosphate
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
HeLa cell
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O
-
 Homo sapiens ADP + phosphate
-
 ?

Synonyms

Synonyms Comment Organism
peroxisomal AAA ATPase complex
-
 Homo sapiens
Pex1
-
 Homo sapiens
PEX6
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction enzyme complex absence results in the selective degradation of the peroxisome. Loss of the enzyme complex does not prevent matrix protein import, but instead causes an upregulation of peroxisome degradation by macroautophagy, or pexophagy. The loss of enzyme complex function in cells results in the accumulation of ubiquitinated PEX5 on the peroxisomal membrane that signals pexophagy Homo sapiens
metabolism the peroxisomal enzyme-complex is required to remove the ubiquitinated form of the shuttling peroxisomal matrix protein receptor, PEX5, from the peroxisomal membrane Homo sapiens
physiological function the peroxisomal enzyme-complex is required for peroxisome quality control Homo sapiens