Protein Ercc6

URN urn:agi-llid:1162
Connectivity 56
Name Ercc6
Description excision repair cross-complementing rodent repair deficiency, complementation group 6
Notes This gene encodes a DNA-binding protein that is important in transcription-coupled excision repair. The protein has ATP-stimulated ATPase activity; there are contradictory publications reporting presence or absence of helicase activity. The protein appears to interact with several transcription and excision repair proteins, and may promote complex formation at repair sites. Mutations in this gene result in Cockayne syndrome type B. [provided by RefSeq]

Pathway nucleotide excision repair pathway

GO Molecular Function nucleotide binding
chromatin binding
hydrolase activity
transcription elongation regulator activity
protein N-terminus binding
protein C-terminus binding
protein complex binding
DNA binding
ATP binding
helicase activity
DNA helicase activity
DNA-dependent ATPase activity

GO Cellular Component soluble fraction
nucleoplasm
nucleolus
transcription elongation factor complex
nucleus

GO Biological Process activation of JUN kinase activity
regulation of transcription
positive regulation of RNA elongation
activation of JNKK activity
DNA damage response, signal transduction resulting in induction of apoptosis
transcription from RNA polymerase II promoter
DNA repair
base-excision repair
pyrimidine dimer repair
transcription-coupled nucleotide-excision repair
response to DNA damage stimulus
response to oxidative stress
response to toxin
response to superoxide
response to gamma radiation
response to X-ray
response to UV
response to UV-B
photoreceptor cell maintenance

Ariadne Ontology Polymerase II transcription
DNA repair

Group Polymerase II transcription
DNA repair
activation of JUN kinase activity
regulation of transcription
positive regulation of RNA elongation
activation of JNKK activity
DNA damage response, signal transduction resulting in induction of apoptosis
transcription from RNA polymerase II promoter
DNA repair
base-excision repair
pyrimidine dimer repair
transcription-coupled nucleotide-excision repair
response to DNA damage stimulus
response to oxidative stress
response to toxin
response to superoxide
response to gamma radiation
response to X-ray
response to UV
response to UV-B
photoreceptor cell maintenance
nucleotide binding
chromatin binding
hydrolase activity
transcription elongation regulator activity
protein N-terminus binding
protein C-terminus binding
protein complex binding
DNA binding
ATP binding
helicase activity
DNA helicase activity
DNA-dependent ATPase activity
soluble fraction
nucleoplasm
nucleolus
transcription elongation factor complex
nucleus

MedScan ID 1162

Hugo ID 3438

Human chromosome position 10q11.23

LocusLink ID 1162
2074
319955
306274

Alias CSB
CKN2
COFS
ARMD5
COFS1
RAD26
DNA excision repair protein ERCC-6
Rad26 homolog
Cockayne syndrome B protein
ATP-dependent helicase ERCC6
cockayne syndrome protein CSB
Cockayne syndrome group B protein
4732403I04
C130058G22Rik
CS group B correcting
C130058G22
Cockayne syndrome 2 (late onset)
cockayne syndrome group b gene
cockayne syndrome II gene
cockayne syndrome II protein
CS group B correcting gene
DNA repair helicase ERCC6
ERC6
ERC6_HUMAN
ERCC VI
Ercc6
ERCCVI
excision repair 6
excision repair cross-complementing rodent repair deficiency, complementation group
excision repair cross-complementing rodent repair deficiency, complementation group 6
Excision repair protein ERCC VI
Excision repair protein ERCC-6
Excision repair protein ERCC-6 (Cockayne syndrome protein CSB)
LOC282965
OTTHUMP00000019581
Rad26 (yeast) homolog

Organism Homo sapiens
Mus musculus
Rattus norvegicus

GenBank ID NC_000010
NM_000124
NP_000115
NT_030059
NW_001837975
NW_924685
NG_009442
AC_000142
AC_000053
AC073366
AL138760
CAH70291
AY204752
AAO13487
CH471187
EAW93094
EAW93097
AB209504
BAD92741
AK094670
AK130100
AK303022
BAG64148
BC127104
AAI27105
CR749388
CAH18238
L04791
AAA52397
Q03468
Q59FF6
NC_000080
NM_001081221
NP_001074690
NT_039606
NW_001030543
AC_000036
AC154412
AK028576
AK040645
AK048416
BAC33330
AK138738
AK139396
BC132447
AAI32448
Q8C851
BC167234
AAI67234
NC_005115
NM_001107296
NW_047469
NW_001084711
AC_000084
CH474046
EDL88913

OMIM ID 609413
214150
133540
278800
211980
600630

Mouse chromosome position 14

GO ID 0005524
0003677
0003678
0008094
0003682
0016787
0000166
0008022
0047485
0032403
0003711
0008630
0007256
0007257
0006284
0045494
0032786
0006290
0045449
0009411
0010224
0010165
0010332
0006979
0000303
0009636
0006366
0006283
0005730
0005654
0005634
0005625
0008023
0006281
0006974
0004386

Rat chromosome position 16p16

Swiss-Prot Accession Q03468
Q59FF6
Q8C851
Q5W0L9
D3DX94

PIR ID A44224

Unigene ID Hs.654449
Mm.318310
Rn.19370

KEGG ID hsa:2074
mmu:319955
rno:306274

EC Number 3.6.1.-

Swiss-Prot ID ERCC6_HUMAN
D3DX94_HUMAN

Cell Localization Nucleus

IPI ID IPI00414779
IPI00844131
IPI00555651
IPI00606873
IPI00358023
IPI00781961

Homologene ID 6279

RGD ID 1311509

MGI ID 1100494