XPGN Xeroderma pigmentosum G N-region
SMART ACC:	SM000485
Description:	domain in nucleases
InterPro ACC:	IPR006085
InterPro abstract:	Xeroderma pigmentosum (XP) [ PUBMED:8160271 ] is a human autosomal recessive disease, characterised by a high incidence of sunlight-induced skin cancer. People's skin cells with this condition are hypersensitive to ultraviolet light, due to defects in the incision step of DNA excision repair. There are a minimum of seven … expand Xeroderma pigmentosum (XP) [ PUBMED:8160271 ] is a human autosomal recessive disease, characterised by a high incidence of sunlight-induced skin cancer. People's skin cells with this condition are hypersensitive to ultraviolet light, due to defects in the incision step of DNA excision repair. There are a minimum of seven genetic complementation groups involved in this pathway: XP-A to XP-G. XP-G is one of the most rare and phenotypically heterogeneous of XP, showing anything from slight to extreme dysfunction in DNA excision repair [ PUBMED:8464724 PUBMED:8206890 ]. XP-G can be corrected by a 133 Kd nuclear protein, XPGC [ PUBMED:8160271 ]. XPGC is an acidic protein that confers normal UV resistance in expressing cells [ PUBMED:8206890 ]. It is a magnesium-dependent, single-strand DNA endonuclease that makes structure-specific endonucleolytic incisions in a DNA substrate containing a duplex region and single-stranded arms [ PUBMED:8206890 PUBMED:8090225 ]. XPGC cleaves one strand of the duplex at the border with the single-stranded region [ PUBMED:8090225 ]. XPG (ERCC-5) belongs to a family of proteins that includes RAD2 from Saccharomyces cerevisiae (Baker's yeast) and rad13 from Schizosaccharomyces pombe (Fission yeast), which are single-stranded DNA endonucleases [ PUBMED:8090225 PUBMED:8247134 PUBMED:32821917 ]; mouse and human FEN-1, a structure-specific endonuclease; RAD2 from fission yeast and RAD27 from budding yeast; fission yeast exo1, a 5'-3' double-stranded DNA exonuclease that may act in a pathway that corrects mismatched base pairs; yeast DHS1, and yeast DIN7. Sequence alignment of this family of proteins reveals that similarities are largely confined to two regions. The first is located at the N-terminal extremity (N-region) and corresponds to the first 95 to 105 amino acids. The second region is internal (I-region) and found towards the C terminus; it spans about 140 residues and contains a highly conserved core of 27 amino acids that includes a conserved pentapeptide (E-A-[DE]-A-[QS]). It is possible that the conserved acidic residues are involved in the catalytic mechanism of DNA excision repair in XPG. The amino acids linking the N-and I-regions are not conserved. This entry represents the N-terminal of XPG. collapse
GO function:	nuclease activity (GO:0004518)
Family alignment:	View the Family alignment or the Alignment consensus sequence
There are 8 355 XPGN domains in 8 349 proteins in SMART's NRDB database.

Taxonomic distribution of proteins containing XPGN domains

The tree below includes only several representative species and genera. The complete taxonomic breakdown of all proteins containing XPGN domains can be accessed here. Click the counts or percentage values to display the corresponding proteins.

Relevant references for this domain

Primary literature for the XPGN domain is listed below. Automatically-derived, secondary literature is also available.

KEGG pathways involving proteins which contain this domain

This information is based on the mapping of SMART genomic protein database to KEGG orthologous groups. Percentages are related to the number of proteins containing a XPGN domain which could be assigned to a KEGG orthologous group, and not all proteins containing XPGN domains. Please note that proteins can be included in multiple pathways, ie. the numbers below will not add to 100%.

KEGG pathways

Some of these pathways are included in the interactive Pathways Explorer overview maps. Select an overview map and click the button below to highlight them in iPath.

KEGG orthologous groups

Some of these KOs are included in the interactive Pathways Explorer overview maps. Select an overview map and click the button below to highlight them in iPath.

3D structures in PDB containing this domain

Links to other resources describing this domain

PROSITE	XPG_2
InterPro	IPR006085
Pfam	XPG_N