parkin coregulated gene protein; molecular chaperone/chaperonin-binding protein; PARK2 coregulated gene protein (PACRG, GLUP)

Function: - suppresses cell death induced by accumulation of unfolded Pael receptor (Pael-R, a substrate of parkin) - facilitates formation of inclusions consisting of Pael-R, molecular chaperones, protein degradation molecules & itself when proteasome is inhibited - may play a role in formation of Lewy bodies & protection of dopaminergic neurons against parkinson disease - forms a large molecular chaperone complex containing heat shock proteins 70 & 90 & chaperonin components - interacts with STIP1, PARK2, GPR37, HSPA8, TCP1/CCT1, CCT2, CCT3, CCT4, CCT5, CCT6A, CCT7, CCT8 Alternative splicing: - named isoforms=2 - may be due to exon skipping Expression: - expressed in all immune tissues, spleen, lymph nodes, thymus, tonsils, leukocyte & bone marrow - also expressed in heart, brain, skeletal muscle, kidney, lung & pancreas - expressed in primary Schwann cells & very weakly by monocyte- derived macrophages the primary host cells of Mycobacterium lepra Pathology: - component of Lewy bodies, intraneuronal inclusions found in brain of patients with Parkinson disease Polymorphism: - involved in susceptibility to leprosy - associated with polymorphisms in the 5'-regulatory region shared by the PARK2 gene Genetics: - linked to PARK2 in a head-to-head arrangement on opposite DNA strands & share a common 5'-flanking promoter region

Related

leprosy (Hansen's disease) PACRG gene parkin gene

Properties

SIZE: entity length = 296 aa MW = 33 kD

Database Correlations

OMIM correlations UniProt Q96M98 Pfam PF10274 Entrez Gene 135138 Kegg hsa:135138

References

UniProt :accession Q96M98