EC Number   |
General Information |
Reference |
|---|
   3.6.4.7 | evolution |
the enzyme belongs to the type II AAA+ ATPases, which by definition contain two conserved nucleotide-binding domains (D1 and D2) in tandem flanked by less conserved N- and C-terminal regions |
734752 |
| 3.6.4.7 | malfunction |
a deletion of peroxisomal Lon results in a specific growth defect on media containing oleic acid as a sole carbon source, conditions which require peroxisomal enzymes of the beta-oxidation pathway, the growth defect is accompanied by the formation of protein aggregates in the peroxisomal matrix |
733421 |
| 3.6.4.7 | malfunction |
a Lon protease deletion strain does not display a growth defect but a decreased viability of the cells |
733421 |
| 3.6.4.7 | malfunction |
a mutation of the conserved Walker A lysine in the D1 domain of Pex1, but not Pex6, dramatically affects the recovery of fully assembled recombinant hexamer |
734485 |
| 3.6.4.7 | 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 |
755919 |
| 3.6.4.7 | malfunction |
in cells of a PLN deletion strain, peroxisomes contain protein aggregates, a major component of which is catalase-peroxidase. Cells of the pln mutant strain contain enhanced levels of catalase-peroxidase protein but reduced catalase-peroxidase enzyme activities. And the absence of Pln results in the formation of protein aggregates in the peroxisomal matrix |
-, 734186 |
| 3.6.4.7 | malfunction |
in fibroblasts from patients defective in Pex1, Pex6 and Pex26, (all of which are required for Pex5 export) Pex5 stability is decreased |
721087 |
| 3.6.4.7 | malfunction |
Lon2 absence leads to accumulation of enzymes in peroxisomes and results in an accelerated peroxisome degradation by pexophagy |
733421 |
| 3.6.4.7 | malfunction |
mutation of the Walker B motif in one D2 domain leads to ATP hydrolysis in the neighbouring domain |
734752 |
| 3.6.4.7 | malfunction |
mutations in Pex1 and Pex6 cause more than 80% of peroxisome biogenesis disorder cases, including Zellweger syndrome |
756699 |
