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

More general terms

• nuclear protein
• protein subunit
• antigen

Additional terms

• polymyositis
• scleroderma (diffuse cutaneous systemic sclerosis)

Component of

• exosome multienzyme ribonuclease complex

Database

• UniProt: http://www.uniprot.org/uniprot/Q01780.html
• OMIM: https://mirror.omim.org/entry/605960