Pathological mutations of the human NDUFS4 gene of the 18-kDa (AQDQ) subunit of complex I affect the expression of the protein and the assembly and function of the complex
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cAMP controls oxygen metabolism in mammalian cellsDysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex IA molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy.NDUFAF7 methylates arginine 85 in the NDUFS2 subunit of human complex IMutations in the NDUFS4 gene of mitochondrial complex I alter stability of the splice variantsProteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) geneMice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.Maneb-induced dopaminergic neuronal death is not affected by loss of mitochondrial complex I activity: results from primary mesencephalic dopaminergic neurons cultured from individual Ndufs4+/+ and Ndufs4-/- mouse embryos.Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson's disease model.Use of (32)P to study dynamics of the mitochondrial phosphoproteomeOpa1 overexpression ameliorates the phenotype of two mitochondrial disease mouse models.Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse BrainGenetic reduction of mitochondrial complex I function does not lead to loss of dopamine neurons in vivoComplex I dysfunction underlies the glycolytic switch in pulmonary hypertensive smooth muscle cells.Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression.Mouse models of mitochondrial complex I dysfunctionMitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat.Constitutive Reprogramming of Fibroblast Mitochondrial Metabolism in Pulmonary Hypertension.TNF-induced mitochondrial damage: a link between mitochondrial complex I activity and left ventricular dysfunction.NDUFS4: creation of a mouse model mimicking a Complex I disorder.Conditional deletion of Ndufs4 in dopaminergic neurons promotes Parkinson's disease-like non-motor symptoms without loss of dopamine neurons.Respiratory chain complex I, a main regulatory target of the cAMP/PKA pathway is defective in different human diseases.Research on plants for the understanding of diseases of nuclear and mitochondrial origin.Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.Accessory subunits of mitochondrial complex I.Regulation of Mitochondrial Complex I Biogenesis in Drosophila Flight Muscles.Hydrogen peroxide induces adaptive response and differential gene expression in human embryo lung fibroblast cells.MITOCHIP assessment of differential gene expression in the skeletal muscle of Ant1 knockout mice: coordinate regulation of OXPHOS, antioxidant, and apoptotic genes.Differential effects of all-trans retinoic acid on the growth of human keratinocytes and mouth carcinoma epidermoid cultures. Involvement of GRIM-19 and complex I of the respiratory chain.Leishmania major abrogates gamma interferon-induced gene expression in human macrophages from a global perspective.Complex I function is defective in complex IV-deficient Caenorhabditis elegans.Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assemblyAssociation between ROS production, swelling and the respirasome integrity in cardiac mitochondria.Proteomic and functional alterations in brain mitochondria from Tg2576 mice occur before amyloid plaque deposition.Streptozotocin-Induced Adaptive Modification of Mitochondrial Supercomplexes in Liver of Wistar Rats and the Protective Effect of Moringa oleifera Lam.
P2860
Q24298085-CEFA5D4B-2F47-4137-A9CF-EB2F8E7ABD0AQ24304454-8009FD90-01F2-4AFD-9A15-662695C7A86AQ24536088-441B6FB9-BF70-493D-BDC4-A1816F501A19Q28115826-85C1F032-F5FD-47FB-BE62-413E589F172EQ28258107-DF980C83-F89B-41CA-B70E-0E4A622BDF09Q28579972-E41FBBD3-94E6-4439-B911-93B04D9D75ABQ30484862-6C2E79AC-6757-4D0A-91DC-D9C8FBF2AD30Q34422161-3D7D5E0D-94A6-413A-81F7-13A5DD5DDD11Q34637864-339C29B6-3097-47EC-BD02-1DB1E425D2F6Q35225791-895D07A7-D7DC-460B-930D-A714B7953BA8Q35689631-BABEF796-F27F-43A9-BA45-DFF7AE0F8EEEQ35908395-1AD610B9-7724-452D-8BE4-99EDA70BD93FQ35912064-5EEF356B-D196-4599-BA44-4326D9E4E18DQ36017200-FFAC3009-81BF-480C-A1D5-DFA8F822CECEQ36184704-988DF10B-E587-44A4-8698-3B727262ACFEQ36428810-43C71C82-774D-43F8-94C9-D57CD1C61C0CQ36937206-CE923BAF-18E3-470F-888F-23EBAB2527A4Q37088266-86A2A2B9-2DA4-4724-9056-5DF9D085B55AQ37326617-6D7DD5F3-32A2-48F6-AA1D-DB48CE6699C0Q37440629-B6872147-DEBE-4D0A-8C02-065B071B0592Q37715032-0BE524D9-982B-4AC3-8C5D-41C891568FB7Q37938299-049E2F4E-14BE-4925-9660-06A9F4623AF0Q38018051-5D0ADFD5-C5DE-4F96-AFD7-D1CB5E574E69Q38078887-D570D391-3B46-4913-AB36-33AA2E2A412CQ38141073-7D45649C-2819-40AD-8055-5A82B67760C5Q38690884-06D6475C-0334-4542-B29D-FD8F0C969BBFQ39367075-35EB8DEC-4E92-40A5-863C-1399F8B546EDQ39444118-DCD7AA05-5061-4009-947C-191B58B09AFDQ40027769-7152BA91-8CB4-42A5-A12A-0A0A2938249CQ40139059-906B0237-D475-43EA-8878-43F5F89F0AE8Q41983183-7E930087-575B-4D7C-83ED-8CEDE75E8B79Q42591168-0188D7B1-BF60-4E75-95AD-D72A9C679B03Q46331933-795ABD2B-54DD-4D8C-98EA-0DCC86768046Q48269112-9D7AB74D-BA5F-42F3-9C31-FBE63E61DB6BQ52311644-E2AB3FBD-0D06-42CB-AB20-F23A9ECB2CEA
P2860
Pathological mutations of the human NDUFS4 gene of the 18-kDa (AQDQ) subunit of complex I affect the expression of the protein and the assembly and function of the complex
description
2003 nî lūn-bûn
@nan
2003 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
Pathological mutations of the ...... ly and function of the complex
@ast
Pathological mutations of the ...... ly and function of the complex
@en
Pathological mutations of the ...... ly and function of the complex
@nl
type
label
Pathological mutations of the ...... ly and function of the complex
@ast
Pathological mutations of the ...... ly and function of the complex
@en
Pathological mutations of the ...... ly and function of the complex
@nl
prefLabel
Pathological mutations of the ...... ly and function of the complex
@ast
Pathological mutations of the ...... ly and function of the complex
@en
Pathological mutations of the ...... ly and function of the complex
@nl
P2093
P2860
P356
P1476
Pathological mutations of the ...... ly and function of the complex
@en
P2093
Damiano Panelli
Jan A Smeitink
Rosanna Tamborra
Rosaria Vergari
Salvatore Scacco
Sandy Budde
Sergio Papa
Vittoria Petruzzella
P2860
P304
P356
10.1074/JBC.M307615200
P407
P577
2003-11-07T00:00:00Z