The exosome is a multi-subunit complex composed of several highly conserved proteins, some of which are 3â€™ to 5â€™ exoribonucleases. The complex is involved in a variety of cellular processes and is responsible for degrading unstable mRNAs that contain AU-rich (ARE) elements in their untranslated 3â€™ region. EXOSC10, also known as PMSCL, PMSCL2, p2, p3, p4, RRP6, Rrp6p, PM-Scl, or PM/Scl-100, is an 885 amino acid protein that contains one HRDC domain and one 3â€™-5â€™ enonuclease domain. Localized to both the cytoplasm and the nucleus, EXOSC10 is part of the post-splicing exosome complex and is involved in mRNA surveillance, mRNA nuclear export and nonsense-mediated decay of mRNAs containing premature stop codons. against EXOSC10 have been found in patients with scleroderma and/or polymyositis (chronic diseases of the skin and muscle, respectively), suggesting that EXOSC10 may be involved in the pathogenesis of these diseases. Two isoforms of EXOSC10 exist due to alternative splicing events.
Synonyms: Autoantigen PM/Scl 2, Exosc10, Exosome component 10, EXOSX_HUMAN, P100 polymyositis scleroderma overlap syndrome associated autoantigen, P100 polymyositis-scleroderma overlap syndrome-associated autoantigen, p2, p3, p4, PM Scl, PM/Scl 100, PM/Scl-100, PMSCL, PMSCL2, Polymyositis/scleroderma autoantigen 100 kDa, Polymyositis/scleroderma autoantigen 2 100 kDa, Polymyositis/scleroderma autoantigen 2, RRP6, Rrp6p.