Disease:Antibodies against PM/SCL are found in patients with polymyositis and/or scleroderma.,Function:Part of a post-splicing multiprotein complex involved in both mRNA nuclear export and mRNA surveillance. Involved in nonsense-mediated decay (NMD) of mRNAs containing premature stop codons. Component of the exosome 3'->5' exoribonuclease complex, a complex that degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions. Required for the 3'processing of the 7S pre-RNA to the mature 5.8S rRNA. Could be an exonuclease (By similarity). Mediates the association of MTR4, C1D and MPP6 wth the exosome and this complex is required for the maturation of 5.8S rRNA. Required for nucleolar localization of C1D.,similarity:Contains 1 3'-5' exonuclease domain.,similarity:Contains 1 HRDC domain.,subunit:Component of the exosome multienzyme ribonuclease complex composed of at least 11 proteins: EXOSC1/CSL4, EXOSC2/RRP4, EXOSC3/RRP40, EXOSC4/RRP41, EXOSC5/RRP46, EXOSC6/MTR3, EXOSC7/RRP42, EXOSC8/RRP43, EXOSC9/Scl-75, DIS3/RRP44, and EXOSC10/Scl-100 (only in the nuclear complex). Found in a mRNA decay complex with RENT1, RENT2 and RENT3B. Also associated with the GTPase Ran (By similarity). Interacts with PTGES2. Interacts with C1D. Forms a heterotrimeric complex with C1D and MPP6 in vitro.,
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