Parkin Ubiquitin Protein Ligase
Parkin Ubiquitin Protein Ligase | ||
---|---|---|
Properties of human protein | ||
Mass / length primary structure | 465 amino acids | |
Secondary to quaternary structure | Heterodimers | |
Isoforms | 6th | |
Identifier | ||
Gene name | PARK2 | |
External IDs | ||
Enzyme classification | ||
EC, category | 6.3.2. , Ligase | |
Response type | Ubiquitinylation of Proteins | |
Substrate | Protein-lys + ubiquitin | |
Products | Protein-lys-ubiquitin | |
Orthologue | ||
human | House mouse | |
Entrez | 5071 | 50873 |
Ensemble | ENSG00000185345 | ENSMUSG00000023826 |
UniProt | O60260 | Q9WVS6 |
Refseq (mRNA) | NM_004562 | NM_016694 |
Refseq (protein) | NP_004553 | NP_057903 |
Gene locus | Chr 6: 161.35 - 162.73 Mb | Chr 17: 10.84 - 12.06 Mb |
PubMed search | 5071 |
50873
|
Parkin is an enzyme in mammals that marks defective proteins in dopaminergic neurons by attaching a ubiquitin residue so that they are transported away and broken down. It belongs to the ubiquitin protein ligases . A defect in this enzyme, caused by mutations in PARK2 - gene for hereditary Parkinson's disease is responsible: The non-degraded proteins accumulate in the nerve cells and ultimately destroy them. In many cases, the onset of the disease is asymptomatic and depends on other factors.
In order to function correctly, Parkin combines with other proteins (UBE2L3, UBE2L6) to form a multi-enzyme complex. It is also part of another protein complex and interacts with several individual proteins. Parkin is localized in the cytosol, but can be recruited from defective mitochondria.
literature
- TM Dawson: Parkin and defective ubiquitination in Parkinson's disease. In: Journal of neural transmission. Supplement. Number 70, 2006, pp. 209-213, ISSN 0303-6995 . PMID 17017531 . (Review).
- T. Yasuda, H. Mochizuki: The regulatory role of? Synuclein and parkin in neuronal cell apoptosis; possible implications for the pathogenesis of Parkinson's disease. In: Apoptosis. Volume 15, Number 11, November 2010, pp. 1312-1321, ISSN 1573-675X . doi : 10.1007 / s10495-010-0486-8 . PMID 20221696 . (Review).
Individual evidence
- ↑ UniProt O60260
- ↑ T. Kitada, S. Asakawa et al. a .: Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. In: Nature. Volume 392, Number 6676, April 1998, pp. 605-608. doi : 10.1038 / 33416 . PMID 9560156 .
- ↑ E. Lohmann, M. Periquet and a .: How much phenotypic variation can be attributed to parkin genotype? In: Annals of neurology. Volume 54, Number 2, August 2003, pp. 176-185, ISSN 0364-5134 . doi : 10.1002 / ana.10613 . PMID 12891670 .
Web links
- VBGerritsen: life's tremors