Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy.
about
Differential production of superoxide by neuronal mitochondriaGenetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystoniaRespiratory chain complex I deficiencyLeber hereditary optic neuropathyMitochondrial optic neuropathies - disease mechanisms and therapeutic strategies.The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.Mitochondrial production of oxygen radical species and the role of Coenzyme Q as an antioxidant.Increase of mitochondrial DNA in blood cells of patients with Leber's hereditary optic neuropathy with 11778 mutationMechanisms of retinal ganglion specific-cell death in Leber hereditary optic neuropathy.Inherited mitochondrial optic neuropathies.Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutationsFunctional analysis of lymphoblast and cybrid mitochondria containing the 3460, 11778, or 14484 Leber's hereditary optic neuropathy mitochondrial DNA mutation.Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy.Major changes in complex I activity in mitochondria from aged rats may not be detected by direct assay of NADH:coenzyme Q reductase.Simultaneous occurrence of the 11778 (ND4) and the 9438 (COX III) mtDNA mutations in Leber hereditary optic neuropathy: molecular, biochemical, and clinical findings.The 14484 ND6 mtDNA mutation in Leber hereditary optic neuropathy does not affect fibroblast complex I activity.The ubiquinone-binding site in NADH:ubiquinone oxidoreductase from Escherichia coli.Novel mitochondrial C15620A variant may modulate the phenotype of mitochondrial G11778A mutation in a Chinese family with Leigh syndrome.Distal genes of the nuo operon of Rhodobacter capsulatus equivalent to the mitochondrial ND subunits are all essential for the biogenesis of the respiratory NADH-ubiquinone oxidoreductase.The 49-kDa subunit of NADH-ubiquinone oxidoreductase (Complex I) is involved in the binding of piericidin and rotenone, two quinone-related inhibitors.Decrease of rotenone inhibition is a sensitive parameter of complex I damage in brain non-synaptic mitochondria of aged rats.Underevaluation of complex I activity by the direct assay of NADH-coenzyme Q reductase in rat liver mitochondria.Inhibitor sensitivity of respiratory complex I in human platelets: a possible biomarker of ageing.Advances in therapeutic strategies for Leber’s hereditary optic neuropathy
P2860
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P2860
Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy.
description
1994 nî lūn-bûn
@nan
1994年の論文
@ja
1994年学术文章
@wuu
1994年学术文章
@zh
1994年学术文章
@zh-cn
1994年学术文章
@zh-hans
1994年学术文章
@zh-my
1994年学术文章
@zh-sg
1994年學術文章
@yue
1994年學術文章
@zh-hant
name
Functional alterations of the ...... s hereditary optic neuropathy.
@en
Functional alterations of the ...... s hereditary optic neuropathy.
@nl
type
label
Functional alterations of the ...... s hereditary optic neuropathy.
@en
Functional alterations of the ...... s hereditary optic neuropathy.
@nl
prefLabel
Functional alterations of the ...... s hereditary optic neuropathy.
@en
Functional alterations of the ...... s hereditary optic neuropathy.
@nl
P2093
P2860
P1433
P1476
Functional alterations of the ...... 's hereditary optic neuropathy
@en
P2093
E Lugaresi
M Degli Esposti
P Montagna
S Sangiorgi
P2860
P304
P356
10.1016/0014-5793(94)00971-6
P407
P577
1994-10-01T00:00:00Z