Catalog Number:

GT15043

Product Type:

Affinity purified

Immunogen Sequence:

Recombinant human Parkin isoform 2

Host:

Goat

Reactivity:

Human

Applications:

Immunohistochemistry, Western Blot, ELISA

Description:

Parkin is involved in protein degradation as a ubiquitin-protein ligase. The PARK2 gene is mutant in autosomal recessive juvenile parkinsonism.

Parkinson’s Disease, the second most common neurodegenerative disease after Alzheimer’s Disease, is characterized by the loss of dopaminergic neurons and the presence of Lewy bodies (comprised of alpha-synuclein and parkin inclusions). Autosomal Recessive Juvenile Parkinsonism (AR-JP) is a recently described form of Parkinson?s Disease that has been linked to a gene that codes for parkin. Parkin, a 52 kDa protein, has a suggested role in the ubiquitin/proteasome pathway for protein degradation. The amino terminus bears sequence homology to ubiquitin while functionally it acts as a RING-type ubiquitin protein ligase (E3) that coordinates the transfer of ubiquitin to substrate proteins, thus targeting them for degradation by the proteasome.

Studies show that parkin interacts with and ubiquitinates the alpha-synuclein interacting protein, synphilin-1. Recent analysis of parkin mutations in AR-JP patients reveals that parkin becomes functionally inactive as an E3 enzyme. This suggests that the loss of function mutation within parkin results in a failure of ubiquitin/proteasome-mediated proteolysis of synphilin-1 and the accumulation of proteins within the cell resulting in neuronal cell death.

Reference:

Kitada T et al. Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature 392:605-8 (1998).

Abbas, N.; Lucking, C. B.; Ricard, S.; Durr, A.; Bonifati, V.; De Michele, G.; Bouley, S.; Vaughan, J. R.; Gasser, T.; Marconi, R.; Broussolle, E.; Brefel-Courbon, C.; and 13 others : A wide variety of mutations in the parkin gene are responsible for autosomal recessive parkinsonism in Europe. Hum. Molec. Genet. 8: 567-574, 1999.

Kitada, T.; Asakawa, S.; Hattori, N.; Matsumine, H.; Yamamura, Y.; Minoshima, S.; Yokochi, M.; Mizuno, Y.; Shimizu, N. : Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature 392: 605-608, 1998.