Parkin Ubiquitin Protein Ligase

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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

  1. UniProt O60260
  2. 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 .
  3. 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 .

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