exosome complex exonuclease RRP45 (polymyositis/scleroderma autoantigen 1, autoantigen PM/Scl 1, polymyositis/scleroderma autoantigen 75 kD, PM/Scl-75, P75 polymyositis-scleroderma overlap syndrome associated autoantigen, EXOSC9, PMSCL1)

Function

• component of the exosome 3'->5' exoribonuclease complex
• also associated with the GTPase Ran (putative)

Structure

belongs to the Rnase PH family

Compartment

cytoplasm, nucleus, nucleolus

Alternative splicing

named isoforms=4

Pathology

• autoantibody in patients with polymyositis & scleroderma or with overlap of these two conditions

Laboratory

• PM-SCL-75 Ab in serum

More general terms

• protein subunit
• nuclear protein
• phosphoprotein

Additional terms

• polymyositis
• scleroderma (diffuse cutaneous systemic sclerosis)

Component of

• exosome multienzyme ribonuclease complex

Database

• OMIM: https://mirror.omim.org/entry/606180
• UniProt: http://www.uniprot.org/uniprot/Q06265.html