Annotation Detail

Information
Associated Genes
ERCC2
Associated Variants
ERCC2 p.Arg683Trp (p.R683W) ( ENST00000391944.8, ENST00000391945.10, ENST00000684407.1 )
ERCC2 p.Arg683Trp (p.R683W) ( ENST00000391944.8, ENST00000391945.10, ENST00000684407.1 )
Associated Disease
Malignant neoplasm of skin
Source Database
DisGeNET
Description
Many XP patients are compound heterozygotes with a causative XPD point mutation R683W and different second mutant alleles, considered null alleles. However, there is marked clinical heterogeneity (including presence or absence of skin cancers or neurological degeneration) in these XPD/R683W patients, thus suggesting a contribution of the second allele.
Pubmed
19934020
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.00481000882732476
Year of publication
2009
Drugs