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Gene Family: Exosome complex (EXOSC)

Also known as : "PM/Scl complex"

Exosome complex: The exosome complex (or PM/Scl complex, often just called the exosome) is a multi-protein complex capable of degrading various types of RNA (ribonucleic acid) molecules. Exosome complexes are found in both eukaryotic cells and archaea, while in bacteria a simpler complex called the degradosome carries out similar functions. The core of the exosome contains a six-membered ring structure to which other proteins are attached. In eukaryotic cells, the exosome complex is present in the cytoplasm, nucleus and especially the nucleolus, although different proteins interact with the exosome complex in these compartments regulating the RNA degradation activity of the complex to substrates specific to these cell compartments. Substrates of the exosome include messenger RNA, ribosomal RNA, and many species of small RNAs. The exosome has an exoribonucleolytic function, meaning it degrades RNA starting at one end (the 3′ end in this case), and in eukaryotes also an endoribonucleolytic function, meaning it cleaves RNA at sites within the molecule. Several proteins in the exosome are the target of autoantibodies in patients with specific autoimmune diseases (especially the PM/Scl overlap syndrome ) and some antimetabolitic chemotherapies for cancer function by blocking the activity of the exosome. In addition, mutations in exosome component 3 cause pontocerebellar hypoplasia and spinal motor neuron disease. [Source: Wikipedia]

Genes contained within the family: 12

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
EXOSC1 exosome component 1 hCsl4p, Csl4p, CSL4, Ski4p, SKI4, CGI-108, p13 10q24.1
EXOSC2 exosome component 2 hRrp4p, Rrp4p, RRP4, p7 9q34.12
EXOSC3 exosome component 3 hRrp40p, Rrp40p, RRP40, CGI-102, p10, hRrp-40 9p13.2
EXOSC4 exosome component 4 hRrp41p, FLJ20591, Rrp41p, RRP41, RRP41A, Ski6p, SKI6, p12A 8q24.3
EXOSC5 exosome component 5 hRrp46p, Rrp46p, RRP46, RRP41B, MGC12901, p12B 19q13.2
EXOSC6 exosome component 6 MTR3, hMtr3p, Mtr3p, EAP4, p11 16q22.1
EXOSC7 exosome component 7 hRrp42p, Rrp42p, RRP42, EAP1, KIAA0116, p8 3p21.31
EXOSC8 exosome component 8 OIP2, RRP43, bA421P11.3, Rrp43p, EAP2, p9, CIP3 13q13.3
EXOSC9 exosome component 9 PMSCL1 PM/Scl-75, Rrp45p, RRP45, p5, p6 4q27
EXOSC10 exosome component 10 PMSCL2 PM-Scl, PM/Scl-100, Rrp6p, RRP6, p2, p3, p4 1p36.22
DIS3 DIS3 homolog, exosome endoribonuclease and 3'-5' exoribonuclease KIAA1008 RRP44, dis3p, EXOSC11 13q21.33
DIS3L DIS3 like exosome 3'-5' exoribonuclease MGC4562, FLJ38088, KIAA1955, DIS3L1 15q22.31