XERODERMA PIGMENTOSUM PROTEIN GROUP G

GWB-63F6E8

2073

8H7

Clone 8H7 is specific for the Xenoderma Pigmentation protein G. Patients with XPG produce truncated proteins encoded by the ERCC5 gene severly lacking endonuclease activity. Patients with XPG are extremely photosensitive presenting with erythrema and blistering often giving rise to skin cancers. Skin fibroblasts are exquisitively sensitive to UV due to severe deficiency in nuceotide excision repair. Histology: LnCap cells.

Phosphate buffered saline

Single-stranded structure-specific DNA endonuclease involved in DNA excision repair. Makes the 3\' incision in DNA nucleotide excision repair (NER). Acts as a cofactor for a DNA glycosylase that removes oxidized pyrimidines from DNA. May also be involved in transcription-coupled repair of this kind of damage in transcription by RNA polymerase II and perhaps in other processes too.

Nucleus.

Belongs to the XPG/RAD2 endonuclease family. XPG subfamily. 1. Evans. E. et al. (1997) Open complex formation around a lesion during nucleotide excision repair provides a structure for cleavage by human XPG protein. 2. O\' Donovan. A. et al. (1994) Isolation of active recombinant XPG protein. a human DNA repair endonuclease. 3. Nouspikel. T. et al. (1997) A common mutational pattern in Cockayne syndrome patients from xeroderma pigmentosum group G: implications for a second XPG function. 4. Caonstaintinou. A. et al. (1999) Conserved residues of human XPG protein important for nuclease activity and function in nucleotide excision repair. 5. Araujo. S. J. . Nigg. E. A. and Wood. R. D. (2001) Strong functional interactions of TFIIH with XPC and XPG in human DNA nucleotide excision repair. without a preassembled repairosome.