Frataxin promotes antioxidant defense in a thiol-dependent manner resulting in diminished malignant transformation in vitro

Human Molecular Genetics

Volumn 11, Issue 7, 2002, Pages 815-821

  Shoichet, Sarah A ^a,g   Bäumer, Anselm T ^a   Stamenkovic, Djordje ^a   Sauer, Heinrich ^a   Pfeiffer, Andreas F H ^b   Kahn, C Ronald ^c   Müller Wieland, Dirk ^d   Richter, Christoph ^e   Ristow, Michael ^b,f  

^a UNIVERSITY OF COLOGNE   (Germany)

^b FREIE UNIVERSITÄT BERLIN   (Germany)

^c HARVARD MEDICAL SCHOOL   (United States)

^d LEIBNIZ CENTER FOR DIABETES RESEARCH AT HEINRICH HEINE UNIVERSITY DÜSSELDORF   (Germany)

^e ETH ZURICH   (Switzerland)

^f GERMAN INSTITUTE OF HUMAN NUTRITION   (Germany)

^g MAX PLANCK INSTITUTE FOR MOLECULAR GENETICS   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

AGAR; FRATAXIN; GLUTATHIONE PEROXIDASE; REACTIVE OXYGEN METABOLITE; THIOL;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANTIOXIDANT ACTIVITY; ARTICLE; ATAXIA; CANCER INCIDENCE; CARCINOGENESIS; CONTROLLED STUDY; ENZYME ACTIVATION; FEMALE; GENE OVEREXPRESSION; GENETIC DISORDER; HUMAN; IN VITRO STUDY; MALIGNANT TRANSFORMATION; MOUSE; NONHUMAN; PRIORITY JOURNAL; TRANSGENE;

ANIMALIA; MUS MUSCULUS;

EID: 0036537486     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/11.7.815     Document Type: Article

References (45)

1. Friedreich Ataxia
2. Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia
3. Mitochondrial impairment of human muscle in Friedreich ataxia in vivo
4. Primary small bowel ganglioneuroblastoma and Friedreich's ataxia
5. Gastric carcinoma in siblings with Friedreich's ataxia
6. Breast cancer in two sisters with Friedreich's ataxia
7. Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
8. Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin
9. Towards a structural understanding of Friedreich's ataxia: The solution structure of frataxin
10. Crystal structure of human frataxin
11. Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
12. Frataxin activates mitochondrial energy conversion and oxidative phosphorylation
13. Increased sensitivity to gamma irradiation of skin fibroblasts in Friedreich's ataxia
14. The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis
15. Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
16. Somatic sequence variation at the Friedreich ataxia locus includes complete contraction of the expanded GAA triplet repeat, significant length variation in serially passaged lymphoblasts and enhanced sequence
17. Oxidative stress in patients with friedreich ataxia
18. Increased levels of plasma malondialdehyde in friedreich ataxia
19. Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
20. Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia
21. Iron-dependent self-assembly of recombinant yeast frataxin: Implications for Friedreich ataxia
22. The mitochondrial proteins Ssq1 and Jac1 are required for the assembly of iron sulfur clusters in mitochondria
23. The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly
24. Effect of idebenone on cardiomyopathy in Friedreich's ataxia: A preliminary study
25. Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia
26. Idebenone in patients with Friedreich ataxia
27. Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone
28. Glutathione in blood of patients with Friedreich's Ataxia
29. Disabled early recruitment of antioxidant defenses in Friedreich's ataxia
30. The multistep nature of cancer
31. Singlet oxygen induces oxidation of cellular DNA
32. Antimutagenesis and anticarcinogenesis, from the past to the future
33. Oxy-radicals and cancer
34. The age of cancer
35. Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation
36. Hydrogen peroxide formation by cells treated with a tumor promotor
37. Superoxide dismutase: Improved assays and an assay applicable to acrylamide gels
38. A spectrophotometric method for measuring the breakdown of hydrogen peroxide by catalase
39. Studies on the quantitative and qualitative characterization of erythrocyte glutathione peroxidase
40. Enzymic method for quantitative determination of nanogram amounts of total and oxidized glutathione: Applications to mammalian blood and other tissues
41. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
42. Growth and activation of human leukaemic cells in vitro and their growth in SCID mouse model