| Name | MELAS |
|---|---|
| Synonyms | MELAS |
| Name | rotenone |
|---|---|
| CAS |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 3132077 | Ichiki T, Tanaka M, Nishikimi M, Suzuki H, Ozawa T, Kobayashi M, Wada Y: Deficiency of subunits of Complex I and mitochondrial encephalomyopathy. . Ann Neurol. 1988 Mar;23(3):287-94. Enzymic activities of the respiratory chain and content of immunochemically detectable subunits in NADH-ubiquinone oxidoreductase (Complex I) were measured in mitochondria from the skeletal muscles of 4 patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The rotenone-sensitive -cytochrome c reductase activity was extremely decreased, ranging from 0% to 27% of the control value. |
2(0,0,0,2) | Details |
| 17276738 | Fujita Y, Ito M, Nozawa Y, Yoneda M, Oshida Y, Tanaka M: CHOP (C/EBP homologous protein) and ASNS (asparagine synthetase) induction in cybrid cells harboring MELAS and NARP mitochondrial DNA mutations. Mitochondrion. 2007 Feb-Apr;7(1-2):80-8. Epub 2006 Dec 3. |
3(0,0,0,3) | Details |
| 3100753 | Kobayashi M, Morishita H, Sugiyama N, Yokochi K, Nakano M, Wada Y, Hotta Y, Terauchi A, Nonaka I: Two cases of NADH-coenzyme Q reductase deficiency: relationship to MELAS syndrome. J Pediatr. 1987 Feb;110(2):223-7. Rotenone-sensitive cytochrome c reductase activities were decreased to 8% (patient 1) and 6% (patient 2) of control values; cytochrome c reductase and cytochrome c oxidase values were within normal limits. These results indicate that both patients have a defect of - reductase of the respiratory chain and that MELAS can be brought about by a defect of - reductase. |
1(0,0,0,1) | Details |
| 9547220 | Patel AJ, Lauritzen I, Lazdunski M, Honore E: Disruption of mitochondrial respiration inhibits volume-regulated anion channels and provokes neuronal cell swelling. J Neurosci. 1998 May 1;18(9):3117-23. VRAC opening is reversibly depressed by hypoxia and by mitochondrial inhibitors such as oligomycin, rotenone, and antimycin A. Moreover, the findings reported in this work may have a particular significance for inherited mitochondrial human diseases, such as mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), which cause brain swelling and edema. |
1(0,0,0,1) | Details |
| 3106581 | Nishizawa M, Tanaka K, Shinozawa K, Kuwabara T, Atsumi T, Miyatake T, Ohama E: A mitochondrial encephalomyopathy with cardiomyopathy. J Neurol Sci. 1987 Apr;78(2):189-201. The clinical features were consistent with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS). The rotenone-sensitive NADH-coenzyme Q reductase activity was markedly decreased in heart, skeletal muscle and liver mitochondria. |
1(0,0,0,1) | Details |
| 17889473 | Hirata K, Akita Y, Povalko N, Nishioka J, Yatsuga S, Matsuishi T, Koga Y: Effect of on synaptosomal mitochondrial function. . Brain Dev. 2008 Apr;30(4):238-45. Epub 2007 Sep 21. Such effects might contribute to alleviation of stroke-like symptoms in MELAS. RESULTS: The entire uptakes of and were inhibited by rotenone (about 30 nmol/mg protein) with dose-dependent manner and showed a plateau at about 70% of total uptake. |
1(0,0,0,1) | Details |
| 16815102 | Cortopassi G, Danielson S, Alemi M, Zhan SS, Tong W, Carelli V, Martinuzzi A, Marzuki S, Majamaa K, Wong A: Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte-specific transcripts. Mitochondrion. 2006 Aug;6(4):161-75. Epub 2006 May 24. To examine the extent to which the transcriptional profile was shared among 5 mitochondrial diseases (LHON, FRDA, MELAS, KSS, and NARP), we microarrayed mutant and control groups in N-tera2, SH-SY5Y, lymphoblasts, fibroblasts, myoblasts, muscle, and osteosarcoma cybrids. Mitochondrial disease activated multiple transcripts of the unfolded protein response (UPR), and of the cell cycle pathway, and low doses of the mitochondrial inhibitor rotenone induced UPR transcripts in the absence of cell death. |
1(0,0,0,1) | Details |