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Gene Family: COP9 signalosome (COPS)

Also known as : "signalosome", "CSN"

COP9 signalosome: COP9 (Constitutive photomorphogenesis 9) signalosome (CSN) is a protein complex with isopeptidase activity. It catalyses the hydrolysis of NEDD8 protein from the cullin subunit of Cullin-RING ubiquitin ligases (CRL). Therefore, it is responsible for CRL deneddylation – at the same time, it is able to bind denedyllated cullin-RING complex and retain them in deactivated form. COP9 signalosome thus serves as a sole deactivator of CRLs. It was found in all eukaryotic organisms including human; it was first discovered in plants. Human COP9 signalosome (total size ~350 kDa ) consists of 8 subunits - CSN1/COPS1, CSN2/COPS2, CSN3/COPS3, CSN4/COPS4, CSN5/COPS5, CSN6/COPS6, CSN7/COPS7A & COPS7B and CSN8/COPS8. All are essential for full function of the complex and mutation in some of them is lethal in mice. [Source: Wikipedia]

Genes contained within the family: 9

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
GPS1 G protein pathway suppressor 1 COPS1, CSN1 17q25.3
COPS2 COP9 signalosome subunit 2 TRIP15, ALIEN, CSN2 15q21.2
COPS3 COP9 signalosome subunit 3 SGN3, CSN3 17p11.2
COPS4 COP9 signalosome subunit 4 CSN4 4q21.22
COPS5 COP9 signalosome subunit 5 JAB1, SGN5, MOV-34, CSN5 8q13.1
COPS6 COP9 signalosome subunit 6 MOV34-34KD, CSN6 7q22.1
COPS7A COP9 signalosome subunit 7A CSN7A 12p13.31
COPS7B COP9 signalosome subunit 7B CSN7B 2q37.1
COPS8 COP9 signalosome subunit 8 COP9, CSN8, MGC1297, SGN8 2q37.3