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Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study
TLDR
The in-vitro data suggest that both iron chelators and antioxidant drugs that may reduce iron are potentially harmful in patients with Friedreich's ataxia and that idebenone protects heart muscle from iron-induced injury. Expand
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.
TLDR
It is shown that neither superoxide dismutases nor the import iron machinery was induced by an endogenous oxidative stress in FRDA patients' fibroblasts in contrast to control cells, suggesting that continuous oxidative damage to iron-sulfur clusters is causative of the mitochondrial deficiency and long term mitochondrial iron overload occurring inFRDA. Expand
Antiproliferative and apoptotic effects in rat and human hepatoma cell cultures of the orally active iron chelator ICL670 compared to CP20: a possible relationship with polyamine metabolism
TLDR
The results suggest that ICL670 has a powerful antitumoural effect and blocks cell proliferation in neoplastic cells by a pathway different from that of CP20 and may constitute a potential adjuvant drug for anticancer therapy. Expand
The new orally active iron chelator ICL670A exhibits a higher antiproliferative effect in human hepatocyte cultures than O-trensox.
TLDR
It is suggested that ICL670A has the most efficient antitumoral effect, blocks cell proliferation by a pathway different of O-trensox and may constitute a potential drug for anticancer therapy. Expand
Biochemical, genetic and immunoblot analyses of 17 patients with an isolated cytochrome c oxidase deficiency.
TLDR
The findings indicate a genetic heterogeneity of COX deficiencies and are suggestive of a prominent involvement of nuclear genes acting on the assembly and maintenance of cytochrome c oxidase. Expand
Characterization of the murine gene for subunit VIIaL of cytochrome c oxidase.
TLDR
Transcriptional activity of the promoter in reporter assays suggested an ubiquitous expression in mouse tissues and the gene for murine cytochrome c oxidase subunit VIIa-L (Cox7aL) and its promoter region were isolated, sequenced and analysed. Expand
Clinical relevance of genetic instability in prostatic cells obtained by prostatic massage in early prostate cancer
TLDR
The presence of LOH of urinary prostatic cells obtained after prostatic massage is significantly associated with CaP on biopsy and may potentially help to identify a set of patients who are candidates for further prostate biopsies. Expand
Cell complementation using Genebridge 4 human:rodent hybrids for physical mapping of novel mitochondrial respiratory chain deficiency genes.
TLDR
A functional complementation approach by growing the patient's fibroblasts in a highly selective medium and transferring human chromosome fragments into RC-deficient fibro Blasts by microcell-mediated transfer makes the physical mapping of the disease genes feasible in some sporadic cases of RC deficiency. Expand
Allelic losses in localized prostate cancer: association with prognostic factors.
TLDR
The relationship between LOH on 8p22 and PNI suggests the presence on this region of a gene involved in epithelium/nerve interaction in prostate cancer, which is associated with PNI but not with the usual prognostic markers, such as PSA and Gleason score. Expand
High frequency of allelic losses in high-grade prostate cancer is associated with biochemical progression after radical prostatectomy.
TLDR
The results suggest that the overall rate of Loh at chromosomal "hot spots" is more likely to be predictive of recurrence than the presence of LOH at any one particular locus. Expand
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