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ScharerDavid Orlando

Scharer, Orlando D.
Schärer Lab.
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XPG: Its products and biological roles

Author(s)
Schaerer, Orlando D.
Issued Date
2008
URI
https://scholarworks.unist.ac.kr/handle/201301/21274
Fulltext
http://link.springer.com/chapter/10.1007%2F978-0-387-09599-8_9
Citation
ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY, v.637, pp.83 - 92
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.
Publisher
SPRINGER-VERLAG BERLIN
ISSN
0065-2598
Keyword
NUCLEOTIDE EXCISION-REPAIRPIGMENTOSUM GROUP-GCOMPLEMENTATION GROUP-GRNA-POLYMERASE-IITRANSCRIPTION-COUPLED REPAIRSHORT LIFE-SPANXERODERMA-PIGMENTOSUMCOCKAYNE-SYNDROMEDNA-REPAIRENDONUCLEASE XPG

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