PACRG
Description
The PACRG (parkin coregulated) is a protein-coding gene located on chromosome 6.
The PACRG gene encodes a protein conserved across metazoans. In vertebrates, it is linked head-to-head with the adjacent parkin gene, associated with autosomal recessive juvenile Parkinson's disease. These genes are co-regulated in various tissues and share a bi-directional promoter. Both genes are associated with leprosy susceptibility. The PACRG protein forms a large molecular complex with chaperones, including heat shock proteins 70 and 90, and chaperonin components. It is a component of Lewy bodies in Parkinson's disease patients and suppresses unfolded Pael receptor-induced neuronal cell death. Multiple transcript variants encoding different isoforms have been found for this gene.
PACRG protein is a microtubule inner protein (MIP) component of the dynein-decorated doublet microtubules (DMTs) in cilia axoneme, essential for motile cilia beating. It safeguards against cell death caused by the accumulation of unfolded Pael receptor (Pael-R, a substrate of Parkin). When proteasome activity is blocked, PACRG facilitates the formation of inclusions comprised of Pael-R, molecular chaperones, protein degradation molecules, and itself. PACRG is potentially involved in Lewy body formation and the defense of dopaminergic neurons against Parkinson's disease.
PACRG is also known as BUG21, GLUP, HAK005771, PACRG2.1, PARK2CRG, pf12.
