From the Molecular Biology to the Gene Therapy of a DNA Repair Syndrome: Fanconi Anemia
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منابع مشابه
Molecular pathogenesis of Fanconi anemia: recent progress.
A rare genetic disease, Fanconi anemia (FA), now attracts broader attention from cancer biologists and basic researchers in the DNA repair and ubiquitin biology fields as well as from hematologists. FA is a chromosome instability syndrome characterized by childhood-onset aplastic anemia, cancer or leukemia susceptibility, and cellular hypersensitivity to DNA crosslinking agents. Identification ...
متن کاملDNA Damage in Leukocytes from Fanconi Anemia (FA) Patients and Heterozygotes Induced by Mitomycin C and Ionizing Radiation as Assessed by the Comet and Comet-FISH Assay
Background: Lymphocytes of Fanconi anemia (FA) show an increased sensitivity to the alkylating agents such as mitomycin C (MMC), but their responses to gamma-irradiation is controversial. The extent of DNA damage in leukocytes of FA patients following irradiation and MMC treatment was studied at cellular and single chromosome level. Methods: DNA damage induced by gamma-rays and MMC was measure...
متن کاملInherent radiosensitivity and its impact on breast cancer chemo-radiotherapy
About 10% of apparently normal individuals are sensitive to clastogenic effects of physico-chemical agents. More than 45% of breast cancer patients’ exhibit elevated radiosensitivity. Although the nature of inherent radiosensitivity is not fully understood, but insufficiency and impaired DNA repair mechanism might be prime cause of radiosensitivity. This is evident from genetically affect...
متن کاملThe Molecular Pathogenesis of Fanconi Anemia: Recent Progress
A rare genetic disease, Fanconi Anemia (FA), now attracts broader attention from cancer biologists and basic researchers in the DNA repair and ubiquitin biology fields as well as from hematologists. FA is a chromosome instability syndrome characterized by childhood-onset aplastic anemia, cancer/leukemia susceptibility, and cellular hypersensitivity to DNA crosslinking agents. Identification of ...
متن کاملA multiprotein nuclear complex connects Fanconi anemia and Bloom syndrome.
Bloom syndrome (BS) is a genetic disorder associated with dwarfism, immunodeficiency, reduced fertility, and an elevated risk of cancer. To investigate the mechanism of this disease, we isolated from human HeLa extracts three complexes containing the helicase defective in BS, BLM. Interestingly, one of the complexes, termed BRAFT, also contains five of the Fanconi anemia (FA) complementation gr...
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تاریخ انتشار 2017