Deficiency of DNA ligase activity in Fanconi's anemia
β Scribed by Monica Hirsch-Kauffmann; Manfred Schweiger; Erwin F. Wagner; Karl Sperling
- Publisher
- Springer
- Year
- 1978
- Tongue
- English
- Weight
- 399 KB
- Volume
- 45
- Category
- Article
- ISSN
- 0340-6717
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β¦ Synopsis
A significant decrease in DNA ligase activity was observed in lymphocytes and fibroblasts of a patient with Fanconi's anemia (FA). This decrease is related to the observed DNA repair deficiency indicated by the delayed closing of repair DNA strands following UV irradiation. Other steps of DNA repair were analyzed in the FA fibroblasts, including endonucleolytic incision of DNA, repair DNA synthesis, and exonucleolytic removal of the photoproducts. No differences were found against control cells. The action of DNA ligase is delayed during replication in the FA cells, as seen by an accumulation of replicative intermediates.
π SIMILAR VOLUMES
Isonicotinic acid hydracide (INH) increases substantially the chromosomal instability in Fanconi's anemia (FA) cells. The same concentrations of INH do not significantly break chromosomes in heterozygous or normal cells. INH does not induce alkylation or cross-links in the DNA, like other mutagens k