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The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V).
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Human Polyclonal NDUFV1 Primary Antibody for WB - ABIN1881573
Morán, Rivera, Sánchez-Aragó, Blázquez, Merinero, Ugalde, Arenas, Cuezva, Martín: Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts. in Biochimica et biophysica acta 2010
Show all 3 Pubmed References
Human Monoclonal NDUFV1 Primary Antibody for RNAi, ELISA - ABIN518257
Sheftel, Stehling, Pierik, Netz, Kerscher, Elsässer, Wittig, Balk, Brandt, Lill: Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I. in Molecular and cellular biology 2009
NDUFV1 knockdown-induced blockage of insulin (show INS Antibodies) signaling was released by protein-tyrosine phosphatase 1B (show PTPN1 Antibodies) knockdown in C2C12 myotubes, and NDUFV1 or SIRT1 (show SIRT1 Antibodies) knockdown did not affect mitochondria biogenesis during C2C12 myogenesis. myogenesis.
The authors use green fluorescent-tagged complex I subunits to determine the kinetics of assembly. Tagged human NDUFV1 can replace endogenous protein in the holo-complex I protein and was also found in complex I subassemblies, suggesting that NDUFV1 may directly incorporate into the holo-enzyme and may also assemble into smaller complexes or in a monomer pool.
The authors clone and characterize the human NADH dehydrogenase (show NDUFA2 Antibodies) [ubiquinone] flavoprotein 1, mitochondrial protein (show COX6B2 Antibodies), also known as NDUFV1. They identify transcription factor binding sites as well as consensus motifs for NADH, flavin mononucleotide, and iron-sulfur binding sites. The human ortholog has high homology to the cow NDUFV1 protein.
The authors provide an overview of the function and assembly of human complex I, also known as NADH dehydrogenase (show NDUFA2 Antibodies) (ubiquinone) 1 alpha subcomplex. They discuss its role in oxidative phosphorylation and the implications of complex I deficiency in disease.
we have used a yeast model system to study the molecular consequences of 16 single amino acid substitutions, classified as pathogenic, in the NDUFV1 subunit of complex I
The presented clinical courses of NDUFV1 and NDUFS1 (show NDUFS1 Antibodies) mutation-based complex I deficiencies are characterized by leukoencephalopathy or early death and expand the already heterogeneous phenotypic spectrum.
small number of putative de novo variants were transmitted from BAP (show PHB2 Antibodies) parents to their ASD (show ARSD Antibodies) offspring, and evidence emerged for a rare duplication CNV at 11p13.3 harboring two putative developmental/neuropsychiatric susceptibility gene(s), GSTP1 (show GSTP1 Antibodies) and NDUFV1.
The results affirm that NDUFV1 mutations are causative of the phenotype in two siblings affected by a diffuse leukodystrophy.
observed 2 consanguinous siblings with early-onset encephalopathy, medulla, brainstem and mesencephalon lesions and death before 8 months of age, caused by a complex I deficiency; identified a missense mutation in the NDUFV1 gene; the mutation, p.Arg386His, affects a highly conserved residue
study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2 (show NDUFS2 Antibodies); 2 siblings were homozygous for previously undescribed R386C mutation in NDUFV1
significant negative-correlation between left ventricular end-diastolic dimension and NDUFV1 production in dilated cardiomyopathy
Sp1 (show PSG1 Antibodies) was abnormally expressed in schizophrenia and its mRNA alteration pattern paralleled that of NDUFV1 and NDUFV2 (show NDUFV2 Antibodies) in schizophrenic patients.
Mutations in the NDUFV1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders.
The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I\; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction\; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
, NADH dehydrogenase ubiquinone flavoprotein 1
, NADH-ubiquinone oxidoreductase
, ubiquinone oxidoreductase
, OXPHOS complex I 51 kDa subunit
, NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
, NADH dehydrogenase flavoprotein 1; 24 kDa (FP)
, NADH-ubiquinone oxidoreductase 51 kDa subunit
, complex I-51kD
, complex I 51 kda subunit
, complex I 51kDa subunit
, complex I, mitochondrial respiratory chain
, mitochondrial NADH dehydrogenase ubiquinone flavoprotein 1
, mitochondrial NADH:ubiquinone oxidoreductase 51 kda subunit
, NADH dehydrogenase flavoprotein 1
, NADH-ubiquinone oxidoreductase 51 kDa subunit, mitochondrial
, mitochondrial complex I subunit NDUFV1