PMID: 641373May 1, 1978Paper

Cockayne's syndrome fibroblasts have increased sensitivity to ultraviolet light but normal rates of unscheduled DNA synthesis

The Journal of Investigative Dermatology
A D AndrewsJ H Robbins

Abstract

Cockayne's syndrome is a form of cachectic dwarfism characterized by acute sun sensitivity and numerous other abnormalities of many organ systems. We studied fibroblasts from 9 Cockayne's syndrome patients to determine if their fibroblasts had abnormal post-ultraviolet light colony-forming ability or abnormal ultraviolet light-induced unscheduled DNA synthesis. The fibroblast strains from all the patients had markedly decreased post-ultraviolet light colony-forming ability in comparison with fibroblasts from control donors. Since this increased ultraviolet light sensitivity is propagable in vitro, it may be a manifestation of, or be closely associated with, the inherited genetic defect of this autosomal recessive disease. However, the patients' fibroblasts had normal rates of ultraviolet light-induced unscheduled DNA synthesis. Thus, unlike the UV sensitivity of DNA excision repair-deficient xeroderma pigmentosum strains, the UV sensitivity of Cockayne's syndrome strains is not related to abnormal DNA excision repair, at least to the extent that this repair process is reflected by rates of ultraviolet light-induced unscheduled DNA synthesis.

Citations

May 1, 1983·Photochemistry and Photobiology·C D LytleJ H Robbins
Aug 1, 1981·Journal of Medical Genetics·R ProopsJ Insley
Sep 25, 2014·Proceedings of the National Academy of Sciences of the United States of America·Yuming WangJesper Q Svejstrup
May 1, 1982·Photochemistry and Photobiology·M F LavinD J Hughes
Jan 1, 1992·American Journal of Medical Genetics·M A Nance, S A Berry
Jun 1, 1980·Clinical and Experimental Dermatology·F Giannelli
Jul 1, 1986·International Journal of Radiation Biology and Related Studies in Physics, Chemistry, and Medicine·L PillidgeR T Johnson
Apr 1, 1984·Journal of Neurology, Neurosurgery, and Psychiatry·J H RobbinsR G Scarpinato
Feb 2, 2002·Nature Reviews. Molecular Cell Biology·Jesper Q Svejstrup
Oct 17, 2006·Pathology International·Satoshi FunakiHiroshi Kitamura
Jan 1, 1988·Journal of Inherited Metabolic Disease·H NishioY Fujiwara
Sep 1, 1988·International Journal of Radiation Biology·J S Rubin
Sep 1, 1985·Journal of Neurology, Neurosurgery, and Psychiatry·J H RobbinsL E Nee
Mar 1, 1985·Jinrui idengaku zasshi. The Japanese journal of human genetics·K TanakaY Okada
Apr 26, 2014·Genetics and Molecular Biology·Carlos Fm Menck, Veridiana Munford
May 27, 2020·Genetics and Molecular Biology·Alexandre Teixeira VessoniCamila Carrião Machado Garcia
Dec 1, 1979·Photochemistry and Photobiology·J H Robbins
Jul 1, 1981·Somatic Cell Genetics·K TanakaY Okada
Jun 1, 1983·International Journal of Radiation Biology and Related Studies in Physics, Chemistry, and Medicine·W P Jeeves, A J Rainbow
May 1, 1991·The British Journal of Dermatology·P G NorrisJ L Hawk
Nov 10, 2001·Annual Review of Genomics and Human Genetics·R E Moses
Aug 11, 2010·Proceedings of the National Academy of Sciences of the United States of America·Susan E CohenGraham C Walker
Dec 24, 2020·The Journal of Investigative Dermatology·Mansoor HussainVilhelm A Bohr
Nov 25, 2020·Seminars in Cell & Developmental Biology·Gustavo Satoru KajitaniCarlos Frederico Martins Menck
Feb 1, 2003·Survey of Ophthalmology·Hélène DollfusAlain Sarasin
Oct 1, 1992·Baillière's Clinical Endocrinology and Metabolism·S Robinson, A Kessling

❮ Previous
Next ❯

Related Concepts

Related Feeds

Cachexia & Brown Fat

Cachexia is a condition associated with progressive weight loss due to severe illness. In cancer patients, it is proposed to occur as a result of tumor-induced energy wasting. Several proteins have been implicated in browning and depletion of white adipose tissue. Here is the latest research on cachexia and brown fat.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.

Cardiac Cachexia

Cardiac cachexia is a syndrome associated with the progressive loss of muscle and fat mass. It most commonly affects patients with heart failure and can significantly decrease the quality of life and survival in these patients. Here is the latest research on cardiac cachexia.