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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
Related
polymyositis
scleroderma (diffuse cutaneous systemic sclerosis)
General
nuclear protein
phosphoprotein
protein subunit
Properties
SIZE: entity length = 439 aa
MW = 49 kD
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: Ser phosphorylation site {S392}
Ser phosphorylation site {S394}
Database Correlations
OMIM 606180
UniProt Q06265
PFAM correlations
References
UniProt :accession Q06265
Component-of
exosome multienzyme ribonuclease complex