Summary

Name ERCC6
Full Name DNA excision repair protein ERCC-6
Synonyms ATP-dependent helicase ERCC6, Cockayne syndrome protein CSB | CSB
Primary ID Q03468
Links - -
Type protein
Relations 8
Function Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes (PubMed:20541997, PubMed:26620705). 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 (PubMed:15548521). It is required for transcription-coupled repair complex formation (PubMed:16916636). It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the sites of RNA polymerase II-blocking lesions (PubMed:16916636). 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 (PubMed:25820262). 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 (PubMed:25820262). 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 (PubMed:25820262). Acts as a chromatin remodeler at DSBs; DNA-dependent ATPase-dependent activity is essential for this function. Remodels chromatin by evicting histones from chromatin flanking DSBs, limiting RIF1 accumulation at DSBs thereby promoting BRCA1-mediated HR (PubMed:29203878). Required for stable recruitment of ELOA and CUL5 to DNA damage sites (PubMed:28292928). Involved in UV-induced translocation of ERCC8 to the nuclear matrix (PubMed:26620705). Essential for neuronal differentiation and neuritogenesis; regulates transcription and chromatin remodeling activities required during neurogenesis (PubMed:24874740).

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▶Modifications Tables

Relations

Regulator Mechanism target score ℹ
+ ABL1up-regulates activity img/direct-activation.png phosphorylation ERCC6 0.274
Publications: 1 Organism: Homo Sapiens
+ ERCC6up-regulates activity img/indirect-activation.png RAD23B 0.469
Publications: 1
+ ERCC6up-regulates activity img/direct-activation.png binding NEIL1 0.352
Publications: 1 Organism: Homo Sapiens
+ ERCC6down-regulates activity img/direct_inhibition.png binding APEX1 0.429
Publications: 1 Organism: Homo Sapiens
+ ERCC8down-regulates quantity by destabilization img/direct_inhibition.png ubiquitination ERCC6 0.601
Publications: 1 Organism: Homo Sapiens
+ DNA_damageup-regulates img/indirect-activation.png ERCC6 0.7
Publications: 1
+ ERCC6form complex img/form-complex.png binding B-WICH complex 0.304
Publications: 1 Organism: Homo Sapiens
+ BRCA1down-regulates quantity by destabilization img/direct_inhibition.png polyubiquitination ERCC6 0.452
Publications: 1 Organism: Homo Sapiens