caa a22 4500 467905630 CHVBK 20180406152833.0 cr unu---uuuuu 170328e20060901xx s 000 0 eng 10.1007/s10735-006-9041-x doi (NATIONALLICENCE)springer-10.1007/s10735-006-9041-x Nucleotide excision repair and cancer [Elektronische Daten] [Diana Leibeling, Petra Laspe, Steffen Emmert] Nucleotide excision repair (NER) is the most versatile and best studied DNA repair system in humans. NER can repair a variety of bulky DNA damages including UV-light induced DNA photoproducts. NER consists of a multistep process in which the DNA lesion is recognized and demarcated by DNA unwinding. Then, a ~28bp DNA damage containing oligonucleotide is excised followed by gap filling using the undamaged DNA strand as a template. The consequences of defective NER are demonstrated by three rare autosomal-rezessive NER-defective syndromes: xeroderma pigmentosum (XP), Cockayne syndrome (CS), and trichothiodystrophy (TTD). XP patients show severe sun sensitivity, freckling in sun exposed skin, and develop skin cancers already during childhood. CS patients exhibit sun sensitivity, severe neurologic abnormalities, and cachectic dwarfism. Clinical symptoms of TTD patients include sun sensitivity, freckling in sun exposed skin areas, and brittle sulfur-deficient hair. In contrast to XP patients, CS and TTD patients are not skin cancer prone. Studying these syndromes can increase the knowledge of skin cancer development including cutaneous melanoma as well as basal and squamous cell carcinoma in general that may lead to new preventional and therapeutic anticancer strategies in the normal population. Springer Science+Business Media B.V., 2006 DNA repair nationallicence Nucleotide excision repair nationallicence Xeroderma pigmentosum nationallicence Cockayne syndrome nationallicence Trichothiodystrophy nationallicence COFS syndrome nationallicence Skin cancer nationallicence Topical therapy with DNA repair enzymes nationallicence Leibeling Diana Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut Laspe Petra Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut Emmert Steffen Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut Journal of Molecular Histology Kluwer Academic Publishers 37/5-7(2006-09-01), 225-238 1567-2379 37:5-7<225 2006 37 10735 https://doi.org/10.1007/s10735-006-9041-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10735-006-9041-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Leibeling Diana Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut NATIONALLICENCE 700 1- Laspe Petra Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut NATIONALLICENCE 700 1- Emmert Steffen Department of Dermatology and Venerology, Georg-August-University Goettingen, Von-Siebold-Strasse 3, 37075, Goettingen, Germany aut NATIONALLICENCE 773 0- Journal of Molecular Histology Kluwer Academic Publishers 37/5-7(2006-09-01), 225-238 1567-2379 37:5-7<225 2006 37 10735 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer