Assembly of mitochondrial complex I and defects in disease.
about
FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathySam50 functions in mitochondrial intermembrane space bridging and biogenesis of respiratory complexesMutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial diseaseTIMMDC1/C3orf1 functions as a membrane-embedded mitochondrial complex I assembly factor through association with the MCIA complexHuman ind1, an iron-sulfur cluster assembly factor for respiratory complex ICharacterization of mitochondrial FOXRED1 in the assembly of respiratory chain complex IMutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial diseaseMultistep assembly of chloroplast NADH dehydrogenase-like subcomplex A requires several nucleus-encoded proteins, including CRR41 and CRR42, in ArabidopsisNDUFA12L mitochondrial complex-I assembly factor: Implications for taupathiesCombined defects in oxidative phosphorylation and fatty acid β-oxidation in mitochondrial diseaseAlterations in c-Myc phenotypes resulting from dynamin-related protein 1 (Drp1)-mediated mitochondrial fission.Atomic structure of the entire mammalian mitochondrial complex I.Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.Conserved and novel functions for Arabidopsis thaliana MIA40 in assembly of proteins in mitochondria and peroxisomesAltering O-linked β-N-acetylglucosamine cycling disrupts mitochondrial function.Defective complex I assembly due to C20orf7 mutations as a new cause of Leigh syndrome.High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiencyMitochondrial function in Antarctic nototheniids with ND6 translocationDJ-1 null dopaminergic neuronal cells exhibit defects in mitochondrial function and structure: involvement of mitochondrial complex I assemblyThe C8ORF38 homologue Sicily is a cytosolic chaperone for a mitochondrial complex I subunit.A three-dimensional topology of complex I inferred from evolutionary correlations.Alkylation sensitivity screens reveal a conserved cross-species functionome.Molecular characterization and expression analysis of NDUFS4 gene in m. longissimus dorsi of Laiwu pig (Sus scrofa).Plasticity of gene expression according to salinity in the testis of broodstock and F1 black-chinned tilapia, Sarotherodon melanotheron heudelotii.A forward genetic screen identifies mutants deficient for mitochondrial complex I assembly in Chlamydomonas reinhardtii.Insights into the composition and assembly of the membrane arm of plant complex I through analysis of subcomplexes in Arabidopsis mutant lines.Mice lacking TR4 nuclear receptor develop mitochondrial myopathy with deficiency in complex I.Trypanosoma brucei mitochondrial respiratome: composition and organization in procyclic formThe complex I subunit NDUFA10 selectively rescues Drosophila pink1 mutants through a mechanism independent of mitophagy.Novel large-range mitochondrial DNA deletions and fatal multisystemic disorder with prominent hepatopathyRedefining the roles of mitochondrial DNA-encoded subunits in respiratory Complex I assemblyLethal mitochondrial cardiomyopathy in a hypomorphic Med30 mouse mutant is ameliorated by ketogenic diet.Maintaining ancient organelles: mitochondrial biogenesis and maturation.Exercise intolerance and developmental delay associated with a novel mitochondrial ND5 mutation.Cell-permeable protein therapy for complex I dysfunctionEnigmatic presence of mitochondrial complex I in Trypanosoma brucei bloodstream forms.Mitochondrial Gene Expression Profiles Are Associated with Maternal Psychosocial Stress in Pregnancy and Infant Temperament.Metabolically induced heteroplasmy shifting and l-arginine treatment reduce the energetic defect in a neuronal-like model of MELAS.L-galactono-1,4-lactone dehydrogenase (GLDH) forms part of three subcomplexes of mitochondrial complex I in Arabidopsis thaliana.A novel immunofluorescent assay to investigate oxidative phosphorylation deficiency in mitochondrial myopathy: understanding mechanisms and improving diagnosis.
P2860
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Assembly of mitochondrial complex I and defects in disease.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on 04 May 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Assembly of mitochondrial complex I and defects in disease.
@en
Assembly of mitochondrial complex I and defects in disease.
@nl
type
label
Assembly of mitochondrial complex I and defects in disease.
@en
Assembly of mitochondrial complex I and defects in disease.
@nl
prefLabel
Assembly of mitochondrial complex I and defects in disease.
@en
Assembly of mitochondrial complex I and defects in disease.
@nl
P50
P1476
Assembly of mitochondrial complex I and defects in disease.
@en
P2093
Michael T Ryan
P356
10.1016/J.BBAMCR.2008.04.015
P407
P577
2008-05-04T00:00:00Z