exosome component 10; polymyositis/scleroderma autoantigen 2; polymyositis/scleroderma autoantigen 100 kD; PM/Scl-100; P100 polymyositis-scleroderma overlap syndrome associated autoantigen (EXOSC10, PMSCL2)

Function: - part of a post-splicing multiprotein complex involved in both mRNA nuclear export & mRNA surveillance - involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons - component of the exosome 3'->5' exoribonuclease complex - could be an exonuclease - mediates the association of MTR4, C1D & MPP6 wth the exosome & this complex is required for the maturation of 5.8S rRNA - required for nucleolar localization of C1D - also associated with the GTPase Ran (putative) - interacts with PTGES2 - forms a heterotrimeric complex with C1D & MPP6 in vitro Structure: - contains 1 3'-5' exonuclease domain - contains 1 HRDC domain Compartment: cytoplasm, nucleus, nucleolus Alternative splicing: named isoforms=2 Pathology: - antibodies against EXOSC10 are found in patients with polymyositis &/or scleroderma

Related

polymyositis scleroderma (diffuse cutaneous systemic sclerosis)

General

antigen nuclear protein protein subunit

Properties

SIZE: MW = 101 kD entity length = 885 aa COMPARTMENT: cell nucleus

Database Correlations

OMIM 605960 UniProt Q01780

References

UniProt :accession Q01780