lmmol · Proteins

DNA excision repair protein ERCC-6

UniProt Q03468 Organism Homo sapiens Gene CSB, ERCC6 EC 3.6.4.-

Also known as: CSB, ERCC6

Function

Essential factor involved in transcription-coupled nucleotide excision repair (TC-NER), a process during which RNA polymerase II-blocking lesions are rapidly removed from the transcribed strand of active genes. Plays a central role in the initiation of the TC-NER process: specifically recognizes and binds RNA polymerase II stalled at a lesion, and mediates recruitment of ERCC8/CSA, initiating DNA damage excision by TFIIH recruitment. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. Acts as a chromatin remodeler at DSBs; DNA-dependent ATPase-dependent activity is essential for this function. Plays an important role in regulating the choice of the DNA double-strand breaks (DSBs) repair pathway and G2/M checkpoint activation; DNA-dependent ATPase activity is essential for this function. Regulates the DNA repair pathway choice by inhibiting non-homologous end joining (NHEJ), thereby promoting the homologous recombination (HR)-mediated repair of DSBs during the S/G2 phases of the cell cycle. Mediates the activation of the ATM- and CHEK2-dependent DNA damage responses thus preventing premature entry of cells into mitosis following the induction of DNA DSBs. Remodels chromatin by evicting histones from chromatin flanking DSBs, limiting RIF1 accumulation at DSBs thereby promoting BRCA1-mediated HR. Required for stable recruitment of ELOA and CUL5 to DNA damage sites. Also involved in UV-induced translocation of ERCC8 to the nuclear matrix. Essential for neuronal differentiation and neuritogenesis; regulates transcription and chromatin remodeling activities required during neurogenesis.

Classification

Family (Pfam)
PF00271 Helicase_C, PF00176 SNF2-rel_dom, PF25875 WHD_Rad26_CSB
InterPro
CC_ERCC-6_N, Helicase_ATP-bd, Helicase_C-like, P-loop_NTPase, SNF2-like_sf, SNF2/RAD54-like_C, SNF2_N, SNF2_RAD54_helicase_repair, WHD_Rad26_CSB-like
Functional cluster
DEAD-Box RNA Helicases & Biogenesis Factors

Experimental structures · PDB · 12

A predicted model is available from AlphaFold.

Gene Ontology · 40

Disease associations

Neighborhood · nearest proteins

CSBCsbAt3g06010smrd-1CKN1KIAA1530ERCC6
Nearest neighbours of DNA excision repair protein ERCC-6: 3 by sequence/function similarity (left); 3 co-cited in the literature (right).

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Literature · 49 cited papers

A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.