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DC Field | Value | Language |
---|---|---|
dc.citation.endPage | 92 | - |
dc.citation.startPage | 83 | - |
dc.citation.title | ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY | - |
dc.citation.volume | 637 | - |
dc.contributor.author | Schaerer, Orlando D. | - |
dc.date.accessioned | 2023-12-22T09:06:39Z | - |
dc.date.available | 2023-12-22T09:06:39Z | - |
dc.date.created | 2017-01-26 | - |
dc.date.issued | 2008 | - |
dc.description.abstract | Xeroderma pigmetosum patients of the complementation group G are rare. One group of XP-G patients displays a rather mild and typical XP phenotype. Mutations in these patients interfere with the function of XPG in the nucleotide excision repair, where it has a structural role in the assembly of the preincision complex and a catalytic role in making the incision 3' to the damaged site in DNA. Another set of XP-G patient is much more severely affected, displaying combined symptoms of xeroderma pigmentosum and Cockayne syndrome, referred to as XP/CS complex. Although the molecular basis leading to the XP/CS complex has not yet been fully established, current evidence suggests that these patients suffer from a mild defect in transcription in addition to a repair defect. Here, the history of how the XPG gene was discovered, the biochemical properties of the XPG protein and the molecular defects found in XP-G patients and mouse models are reviewed. | - |
dc.identifier.bibliographicCitation | ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY, v.637, pp.83 - 92 | - |
dc.identifier.issn | 0065-2598 | - |
dc.identifier.scopusid | 2-s2.0-60549092333 | - |
dc.identifier.uri | https://scholarworks.unist.ac.kr/handle/201301/21274 | - |
dc.identifier.url | http://link.springer.com/chapter/10.1007%2F978-0-387-09599-8_9 | - |
dc.identifier.wosid | 000258488100010 | - |
dc.language | 영어 | - |
dc.publisher | SPRINGER-VERLAG BERLIN | - |
dc.title | XPG: Its products and biological roles | - |
dc.type | Article | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.subject.keywordPlus | NUCLEOTIDE EXCISION-REPAIR | - |
dc.subject.keywordPlus | PIGMENTOSUM GROUP-G | - |
dc.subject.keywordPlus | COMPLEMENTATION GROUP-G | - |
dc.subject.keywordPlus | RNA-POLYMERASE-II | - |
dc.subject.keywordPlus | TRANSCRIPTION-COUPLED REPAIR | - |
dc.subject.keywordPlus | SHORT LIFE-SPAN | - |
dc.subject.keywordPlus | XERODERMA-PIGMENTOSUM | - |
dc.subject.keywordPlus | COCKAYNE-SYNDROME | - |
dc.subject.keywordPlus | DNA-REPAIR | - |
dc.subject.keywordPlus | ENDONUCLEASE XPG | - |
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