|
KMID : 0613320020080030069
|
|
Journal of the Korean Bone and Joint Tumor Soceity
2002 Volume.8 No. 3 p.69 ~ p.75
|
|
|
The Musculoskeletal Tumors of Werner
|
|
Lee Sang-Hoon
Kim Han-Soo
Suh Sung-Wook
Yoo Kwang-Hyun
|
|
|
Abstract
|
|
|
|
Werner´s syndrome is a rare autosomal recessive disorder manifesting as premature aging. It is also known to be characterized by a high frequency of malignant tumors, especially sarcomas. However, Werner´s syndrome may be not only a premature aging disease but also a cancer syndrome, because the malignant tumors in these patients are different from those of normal population with respect to involved site, histological type, and age of onset. Recent studies found Werner´s syndrome was caused by a mutation of Werner helicase suggesting that WRN helicase may participate in metabolism and repair of DNA. And a dysfunction of WRN helicase may induce the genomic instability causing somatic mutations. Further studies of Werner´s syndrome associated with sarcoma might give much informations about the normal aging process and the pathogenesis of sarcomas.
|
|
|
KEYWORD
|
|
|
Werner
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
|