| Name | LHON |
|---|---|
| Synonyms | LHON |
| Name | rotenone |
|---|---|
| CAS |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 11854175 | Wong A, Cavelier L, Collins-Schramm HE, Seldin MF, McGrogan M, Savontaus ML, Cortopassi GA: Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells. Hum Mol Genet. 2002 Feb 15;11(4):431-8. Differentiation of the cells to the neuronal form also resulted in significant increases in ROS production in the LHON-NT2 neurons versus controls, which is abolished by rotenone, a specific inhibitor of Complex I. |
36(0,1,1,6) | Details |
| 7926004 | Degli Esposti M, Carelli V, Ghelli A, Ratta M, Crimi M, Sangiorgi S, Montagna P, Lenaz G, Lugaresi E, Cortelli P: Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy. FEBS Lett. 1994 Oct 3;352(3):375-9. We report that this amino acid substitution alters the affinity of complex I for the substrate and induces resistance towards its potent inhibitor rotenone in mitochondria of LHON patients. |
32(0,1,1,2) | Details |
| 18615737 | Sala G, Trombin F, Beretta S, Tremolizzo L, Presutto P, Montopoli M, Fantin M, Martinuzzi A, Carelli V, Ferrarese C: Antioxidants partially restore transport defect in leber hereditary optic neuropathy cybrids. J Neurosci Res. 2008 Nov 15;86(15):3331-7. Leber hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by visual loss resulting from retinal ganglion cell degeneration. Rotenone, a classic complex I inhibitor, did not worsen the uptake defect present in LHON cybrids under basal conditions but significantly reduced transport in control cybrids. |
5(0,0,0,5) | Details |
| 18235013 | Ghelli A, Porcelli AM, Zanna C, Martinuzzi A, Carelli V, Rugolo M: Protection against oxidant-induced apoptosis by exogenous in Leber hereditary optic neuropathy cybrids. Invest Ophthalmol Vis Sci. 2008 Feb;49(2):671-6. METHODS: Cybrids bearing one of the three most common LHON pathogenic mutations (11778/ND4, 3460/ND1, 14484/ND6) were incubated with two compounds known to induce oxidative injury, tert-butyl hydroperoxide (t-BH) and rotenone. |
14(0,0,2,4) | Details |
| 15342361 | Beretta S, Mattavelli L, Sala G, Tremolizzo L, Schapira AH, Martinuzzi A, Carelli V, Ferrarese C: Leber hereditary optic neuropathy mtDNA mutations disrupt transport in cybrid cell lines. Brain. 2004 Oct;127(Pt 10):2183-92. Epub 2004 Sep 1. Leber hereditary optic neuropathy (LHON) is a maternally inherited form of retinal ganglion cell degeneration leading to optic atrophy which is caused by point mutations in the mitochondrial genome (mtDNA). |
6(0,0,0,6) | Details |
| 15337616 | Kaplanova V, Zeman J, Hansikova H, Cerna L, Houst'kova H, Misovicova N, Houstek J: Segregation pattern and biochemical effect of the G3460A mtDNA mutation in 27 members of LHON family. J Neurol Sci. 2004 Aug 30;223(2):149-55. |
4(0,0,0,4) | Details |
| 16959493 | Beretta S, Wood JP, Derham B, Sala G, Tremolizzo L, Ferrarese C, Osborne NN: Partial mitochondrial complex I inhibition induces oxidative damage and perturbs transport in primary retinal cultures. Neurobiol Dis. 2006 Nov;24(2):308-17. Epub 2006 Sep 7. Relevance to Leber Hereditary Optic Neuropathy (LHON).. Primary rat retinal cultures were exposed to increasing concentrations of rotenone to titrate complex I inhibition. |
4(0,0,0,4) | Details |
| 10072046 | Carelli V, Ghelli A, Bucchi L, Montagna P, De Negri A, Leuzzi V, Carducci C, Lenaz G, Lugaresi E, Degli Esposti M: Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy. Ann Neurol. 1999 Mar;45(3):320-8. We report the effect on complex I function of the 14484 Leber's hereditary optic neuropathy (LHON) mutation affecting the ND6 subunit gene. |
3(0,0,0,3) | Details |
| 18427623 | Levin LA: Mechanisms of retinal ganglion specific-cell death in Leber hereditary optic neuropathy. Trans Am Ophthalmol Soc. 2007;105:379-91. PURPOSE: Leber hereditary optic neuropathy (LHON) results from point mutations in mitochondrial DNA (mtDNA) present in all cells but is only manifested in retinal ganglion cells (RGCs). Rotenone significantly increased the rate of production in brain but not RGC-5 mitochondria. |
3(0,0,0,3) | Details |
| 19233273 | Silva JM, Wong A, Carelli V, Cortopassi GA: Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia. Neurobiol Dis. 2009 May;34(2):357-65. Epub 2009 Feb 20. Maternal inheritance of a pathogenic point mutation within complex I of the mitochondrial genome causes Leber's hereditary optic neuropathy (LHON), resulting in the neurodegeneration and demyelination of the optic nerve. Therefore, we wanted to determine whether mitochondrial dysfunction induced by complex I inhibition with rotenone can activate the ISR, specifically by the ER kinase PERK, in oligodendroglial cells. |
3(0,0,0,3) | Details |
| 18182110 | Hoegger MJ, Lieven CJ, Levin LA: Differential production of by neuronal mitochondria. . BMC Neurosci. 2008 Jan 8;9:4. For example, Leber's hereditary optic neuropathy (LHON) results from one of three point mutations mtDNA coding for complex I components, but is only manifested in retinal ganglion cells (RGCs), a central neuron contained within the retina. Cerebral but not RGC-5 or neuroblastoma cells increased production in response to the complex I inhibitor rotenone, while neuroblastoma but not cerebral or RGC-5 cells dramatically decreased production in response to the complex III inhibitor antimycin A. |
2(0,0,0,2) | Details |
| 9266534 | Ghelli A, Degli Esposti M, Carelli V, Lenaz G: Changes in mitochondrial complex I activity and binding site in Leber's hereditary optic neuropathy (LHON). Mol Aspects Med. 1997;18 Suppl:S263-7. Both 11778/ND4 and 3460/ND1 mutations induced rotenone resistance and 11778/ND4 showed an increased K (m) for -2 with respect to the control group. |
2(0,0,0,2) | Details |
| 9718301 | Zickermann V, Barquera B, Wikstrom M, Finel M: Analysis of the pathogenic human mitochondrial mutation ND1/3460, and mutations of strictly conserved residues in its vicinity, using the bacterium Paracoccus denitrificans. Biochemistry. 1998 Aug 25;37(34):11792-6. The human mitochondrial ND1/3460 mutation changes Ala52 to in the ND1 subunit of Complex I, and causes Leber's hereditary optic neuropathy (LHON) [Huoponen et al. (1991) Am. The enzymatic activity of the mutants in the presence of hexammineruthenium (rotenone-insensitive) and (rotenone-sensitive) were assayed. |
2(0,0,0,2) | Details |
| 1959619 | Majander A, Huoponen K, Savontaus ML, Nikoskelainen E, Wikstrom M: Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON). FEBS Lett. 1991 Nov 4;292(1-2):289-92. The ND1/3460 mutation exhibits 80% reduction in rotenone-sensitive and -dependent electron transfer activity, whereas the proximal NADH dehydrogenase activity of the Complex is unaffected. |
2(0,0,0,2) | 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 |
| 17320357 | Park JS, Li YF, Bai Y: Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation. Biochim Biophys Acta. 2007 May;1772(5):533-42. Epub 2007 Jan 26. G11778A in the subunit ND4 gene of NADH dehydrogenase complex is the most common primary mutation found in Leber's hereditary optic neuropathy (LHON) patients. In transformant cell lines, LeNDI1-1 and -2, total and complex I-dependent respiration were fully restored and largely resistant to complex I inhibitor, rotenone, indicating a dominant role of NDI1 in the transfer of electrons in the host cells. |
1(0,0,0,1) | Details |
| 9191778 | Carelli V, Ghelli A, Ratta M, Bacchilega E, Sangiorgi S, Mancini R, Leuzzi V, Cortelli P, Montagna P, Lugaresi E, Degli Esposti M: Leber's hereditary optic neuropathy: biochemical effect of 11778/ND4 and 3460/ND1 mutations and correlation with the mitochondrial genotype. Neurology. 1997 Jun;48(6):1623-32. To clarify the bioenergetic relevance of mtDNA mutations in Leber's hereditary optic neuropathy (LHON), we investigated affected individuals and healthy carriers from six Italian LHON families harboring the 11778/ND4 and the 3460/ND1 mtDNA mutations. The enzymatic activities of mitochondrial complex I and its sensitivity to the potent inhibitors rotenone and rolliniastatin-2 were studied in mitochondrial particles from platelets, in correlation with mtDNA analysis of platelets and leukocytes. |
1(0,0,0,1) | Details |
| 15505787 | Valentino ML, Barboni P, Ghelli A, Bucchi L, Rengo C, Achilli A, Torroni A, Lugaresi A, Lodi R, Barbiroli B, Dotti M, Federico A, Baruzzi A, Carelli V: The ND1 gene of complex I is a mutational hot spot for Leber's hereditary optic neuropathy. Ann Neurol. 2004 Nov;56(5):631-41. Biochemical investigations in platelets showed partially insensitive complex I to rotenone inhibition. We conclude that the 3733G--> A transition is a novel cause of LHON and, after those at positions 3460 and 4171, is the third ND1 mutation to be identified in multiple unrelated families. |
2(0,0,0,2) | Details |
| 12379308 | Carelli V, Vergani L, Bernazzi B, Zampieron C, Bucchi L, Valentino M, Rengo C, Torroni A, Martinuzzi A: Respiratory function in cybrid cell lines carrying European mtDNA haplogroups: implications for Leber's hereditary optic neuropathy. Biochim Biophys Acta. 2002 Oct 9;1588(1):7-14. The possibility that some combinations of mtDNA polymorphisms, previously associated with Leber's hereditary optic neuropathy (LHON), may affect mitochondrial respiratory function was tested in osteosarcoma-derived transmitochondrial cytoplasmic hybrids (cybrids). |
2(0,0,0,2) | Details |
| 11695835 | Chomyn A: Mitochondrial genetic control of assembly and function of complex I in mammalian cells. J Bioenerg Biomembr. 2001 Jun;33(3):251-7. More recently, we carried out a biochemical, molecular, and cellular analysis of a mutation in the gene for one of these subunits, ND4, that causes Leber's hereditary optic neuropathy (LHON). Subsequently, we isolated several mutants affected in one or another of the mtDNA-encoded subunits of complex I by exposing established cell lines to high concentrations of rotenone. |
1(0,0,0,1) | Details |
| 10426140 | Cock HR, Cooper JM, Schapira AH: Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy. J Neurol Sci. 1999 May 1;165(1):10-7. A 60% rotenone-induced decrease in complex I activity was shown to reduce ATP synthesis in normal fibroblasts, indicating that this level of complex I activity was below the threshold required to affect ATP synthesis. |
0(0,0,0,0) | Details |