| Gene Symbol |
ERCC8
|
| Entrez Gene |
1161
|
| Alt Symbol |
CKN1, CSA, UVSS2
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
excision repair cross-complementation group 8
|
| Other Description |
Cockayne syndrome WD-repeat protein CSA|DNA excision repair protein ERCC-8|cockayne syndrome WD repeat protein CSA|excision repair cross-complementing rodent repair deficiency, complementation group 8
|
| Swissprots |
Q13216 Q6FHX5 Q96GB9 B2RB64
|
| Accessions |
AAO21128 EAW55003 EAW55004 EAW55005 Q13216 AK022657 AK056931 BAG51829 AK226129 AK290726 BAF83415 AK294856 BAG57960 AK304610 BAG65395 AK314511 BAG37111 AL691658 BC009793 AAH09793 BC093802 BC112227 BT020021 AAV38824 CR536563 CAG38800 DA169067 U28413 AAA82605 NM_000082 NP_000073 NM_001007233 NP_001007234 NM_001007234 NP_001007235 NM_001290285 NP_001277214
|
| Function |
Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitment of XAB2, HMGN1 and TCEA1/TFIIS to a transcription- coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes. {ECO:0000269|PubMed:16751180, ECO:0000269|PubMed:16916636, ECO:0000269|PubMed:16964240}.
|
| Subcellular Location |
Nucleus {ECO:0000305}.
|
| Top Pathways |
Ubiquitin mediated proteolysis, Nucleotide excision repair
|