Annotation Detail

Information
Associated Genes
XPC
Associated Variants
XPC p.Pro334Leu (p.P334L) ( ENST00000285021.12 )
XPC p.Pro334Arg (p.P334R) ( ENST00000285021.12 )
XPC p.Pro334His (p.P334H) ( ENST00000285021.12 )
OGG1 c.948+942T>G, CAMK1 p.His334Pro (p.H334P) ( ENST00000302008.12, ENST00000302036.12, ENST00000352937.6, ENST00000383826.9, ENST00000449570.6, ENST00000707074.1, ENST00000256460.8 )
XPC p.Pro334Leu (p.P334L) ( ENST00000285021.12 )
XPC p.Pro334Arg (p.P334R) ( ENST00000285021.12 )
XPC p.Pro334His (p.P334H) ( ENST00000285021.12 )
OGG1 c.948+942T>G, CAMK1 p.His334Pro (p.H334P) ( ENST00000302008.12, ENST00000302036.12, ENST00000352937.6, ENST00000383826.9, ENST00000449570.6, ENST00000707074.1, ENST00000256460.8 )
Associated Disease
xeroderma pigmentosum
Source Database
DisGeNET
Description
In an effort to understand the severity of XP-C phenotypes, we also demonstrated that single mutations in XPC perturb other repair processes, such as base excision repair (e.g., the P334H mutation prevents the stimulation of Ogg1 glycosylase because it thwarts the interaction between XPC and Ogg1), thereby leading to a deeper understanding of the molecular repair defect of the XP-C patients.
Pubmed
18809580
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.0214935655026647
Year of publication
2008
Drugs