g-interferon in mice with a targeted disruption of the Fanconi anemia defect in connection with oxygen and iron sensitivity in Fanconi
ثبت نشده
چکیده
GC, Grompe M: Germ cell defects and hematopoietic sensitivity to 11. Poot M, Gross O, Epe B, Pflaum M, Hoehn H: Cell cycle g-interferon in mice with a targeted disruption of the Fanconi anemia defect in connection with oxygen and iron sensitivity in Fanconi C gene. Blood 88:49, 1996 anemia lymphoblastoid cells. Exp Cell Res 222:262, 1996 2. Saito H, Hammond AT, Moses RE: Hypersensitivity to oxygen 12. Joenje H, Youssoufian H, Kruyt FAE, dos Santos C, Wevrick is a uniform and secondary defect in Fanconi anemia cells. Mutat R, Buchwald M: Expression of the Fanconi anemia gene FAC in Res 294:255, 1993 human cell lines: Lack of effect of oxygen tension. Blood Cells Mol 3. Liu JM, Buchwald M, Walsh CE, Young NS: Fanconi anemia Dis 21:182, 1995 and novel strategies for therapy. Blood 84:3995, 1994 13. Rumyantsev AG, Samochatova EV, Afanas’ev IB, Korkina 4. Korkina LG, Samochatova EV, Maschan AA, Suslova TB, LG, Suslova TB, Cheremisina ZP, Maschan AA, Durnev AD, Lurye Cheremisina ZP, Afanas’ev IB: Release of active oxygen radicals BL: The role of free oxygen radicals in the pathogenesis of Fanconi’s by leukocytes of Fanconi’s anemia patients. J Leukoc Biol 52:357, anemia (in Russian). Ter Arkh 61:32, 1989 1992 14. Savoia A, Zatterale A, Del Principe D, Joenje H: Fanconi 5. Nordenson I: Effect of superoxide dismutase and catalase on anaemia in Italy: High prevalence of complementation group A in spontaneously occurring chromosome breaks in patients with Fantwo geographic clusters. Hum Genet 97:599, 1996 coni’s anemia. Hereditas 86:147, 1977 15. Degan P, Bonassi S, De Caterina M, Korkina LG, Pinto L, 6. Raj AS, Heddle JA: The effect of superoxide dismutase, cataScopacasa F, Zatterale A, Calzone R, Pagano G: In vivo accumulalase and L-cysteine on spontaneous and on mitomycin C induced tion of 8-hydroxy-2-deoxyguanosine in DNA correlates with release chromosomal breakage in Fanconi’s anemia and normal fibroblasts of reactive oxygen species in Fanconi’s anaemia families. Carcinoas measured by the micronucleus method. Mutat Res 78:59, 1980 genesis 16:735, 1995 7. Joenje H, Arwert F, Eriksson AW, de Koning H, Oostra: AB 16. Takeuchi T, Morimoto K: Increased formation of 8-hydroxyOxygen-dependence of chromosomal aberrations in Fanconi’s anaedeoxyguanosine, an oxidative DNA damage, in lymphoblasts from mia. Nature 290:142, 1981 Fanconi’s anemia patients due to possible catalase deficiency. Carci8. Dallapiccola B, Porfirio B, Mokini V, Alimena G, Isacchi G, nogenesis 14:1115, 1993 Gandini E: Effect of oxidants and antioxidants on chromosomal 17. Emerit I, Levy A, Pagano G, Pinto L, Calzone R, Zatterale breakage in Fanconi’s anemia lymphocytes. Hum Genet 69:62, 1985 A: Transferable clastogenic activity in plasma from patients with 9. Joenje H, Gille JJP: Oxygen metabolism and chromosomal FA. Hum Genet 96:14, 1995 breakage in Fanconi’s anemia, in Schroeder-Kurt TM, Auerbach 18. Schulz JC, Shahidi NT: Tumor necrosis factor-a overproducAD, Obe G (eds): Fanconi Anemia: Clinical, Cytogenetic and Expertion in Fanconi’s anemia. Am J Hematol 42:196, 1993 imental Aspects, Berlin, Germany, Springer, 1989, p 174 19. Gordon-Smith EC, Rutherford TR: Fanconi anemia: Constitu10. Schindler D, Hoehn H: Fanconi anemia mutation causes cellular susceptibility to ambient oxygen. Am J Hum Genet 43:429, 1988 tional aplastic anemia. Semin Hematol 28:104, 1991
منابع مشابه
Germ cell defects and hematopoietic hypersensitivity to gamma-interferon in mice with a targeted disruption of the Fanconi anemia C gene.
Fanconi anemia (FA) is an autosomal recessive chromosome instability syndrome characterized by progressive bone marrow (BM) failure, skeletal defects, and increased susceptibility to malignancy. FA cells are hypersensitive to DNA cross-linking agents, oxygen and have cell cycle abnormalities. To develop an animal model of the disease we generated mice homozygous for a targeted deletion of exon ...
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 کاملFrequency of Hypothyroidism in Fanconi Anemia
Background: Fanconi anemia (FA) is a rare, autosomal recessive (AR) and multifactorial disorder. A high prevalence of FA observed in Iran is perhaps due to the high rate of consanguineous marriages. This study investigates the extent of short stature in patients with FA, the frequency of hypothyroidism in FA and the correlation between height and hypothyroidism. Methods: Eighteen patients with ...
متن کاملFanconi Anemia Concurrent with an Unusual Thumb Polydactyly: A Case Report
This case report presents a case of Fanconi’s Anemia with an unusual thumb polydactyly in a 2-year old boy. The extra thumb had no nail, nail bed and distal phalanx. The extra thumb had no active motion.The duplication of the thumb occurred at the carpometacarpal joint but its morphology did not match with any classification described for thumb polydactyly. Although his thumb polydactyly was ...
متن کاملTargeted disruption of the murine Fanconi anemia gene, Fancg/Xrcc9.
Fanconi anemia (FA) is a human autosomal recessive cancer susceptibility disorder characterized by cellular sensitivity to mitomycin C and ionizing radiation. Six FA genes (corresponding to subtypes A, C, D2, E, F, and G) have been cloned, and the encoded FA proteins interact in a common cellular pathway. To further understand the in vivo role of one of these human genes (FANCG), we generated a...
متن کامل