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NADH Dehydrogenase (Ubiquinone) Flavoprotein 1, 51kDa Proteins (NDUFV1)

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). Additionally we are shipping NDUFV1 Antibodies (71) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
NDUFV1 17995 Q91YT0
Rat NDUFV1 NDUFV1 293655  
NDUFV1 4723 P49821
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Top NDUFV1 Proteins at antibodies-online.com

Showing 8 out of 9 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 2 μg Log in to see 9 Days
$333.33
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$3,243.17
Details
Yeast Pongo pygmaeus His tag   1 mg Log in to see 56 to 66 Days
$3,243.17
Details
Yeast Pan troglodytes His tag   1 mg Log in to see 56 to 66 Days
$3,243.17
Details
Yeast Macaca fascicularis His tag   1 mg Log in to see 56 to 66 Days
$3,243.17
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details

NDUFV1 Proteins by Origin and Source

Origin Expressed in Conjugate
Mouse (Murine)

Human , ,
,

More Proteins for NADH Dehydrogenase (Ubiquinone) Flavoprotein 1, 51kDa (NDUFV1) Interaction Partners

Mouse (Murine) NADH Dehydrogenase (Ubiquinone) Flavoprotein 1, 51kDa (NDUFV1) interaction partners

  1. NDUFV1 knockdown-induced blockage of insulin (show INS Proteins) signaling was released by protein-tyrosine phosphatase 1B (show PTPN1 Proteins) knockdown in C2C12 myotubes, and NDUFV1 or SIRT1 (show SIRT1 Proteins) knockdown did not affect mitochondria biogenesis during C2C12 myogenesis. myogenesis.

  2. 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.

  3. The authors clone and characterize the human NADH dehydrogenase (show NDUFA2 Proteins) [ubiquinone] flavoprotein 1, mitochondrial protein (show COX6B2 Proteins), 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.

  4. The authors provide an overview of the function and assembly of human complex I, also known as NADH dehydrogenase (show NDUFA2 Proteins) (ubiquinone) 1 alpha subcomplex. They discuss its role in oxidative phosphorylation and the implications of complex I deficiency in disease.

Human NADH Dehydrogenase (Ubiquinone) Flavoprotein 1, 51kDa (NDUFV1) interaction partners

  1. 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

  2. The presented clinical courses of NDUFV1 and NDUFS1 (show NDUFS1 Proteins) mutation-based complex I deficiencies are characterized by leukoencephalopathy or early death and expand the already heterogeneous phenotypic spectrum.

  3. small number of putative de novo variants were transmitted from BAP (show PHB2 Proteins) parents to their ASD (show ARSD Proteins) offspring, and evidence emerged for a rare duplication CNV at 11p13.3 harboring two putative developmental/neuropsychiatric susceptibility gene(s), GSTP1 (show GSTP1 Proteins) and NDUFV1.

  4. The results affirm that NDUFV1 mutations are causative of the phenotype in two siblings affected by a diffuse leukodystrophy.

  5. 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

  6. study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2 (show NDUFS2 Proteins); 2 siblings were homozygous for previously undescribed R386C mutation in NDUFV1

  7. significant negative-correlation between left ventricular end-diastolic dimension and NDUFV1 production in dilated cardiomyopathy

  8. Sp1 (show PSG1 Proteins) was abnormally expressed in schizophrenia and its mRNA alteration pattern paralleled that of NDUFV1 and NDUFV2 (show NDUFV2 Proteins) in schizophrenic patients.

  9. Mutations in the NDUFV1 gene is linked to a delayed mitochondrial network recovery in OXPHOS disorders.

NDUFV1 Protein Profile

Protein Summary

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.

Gene names and symbols associated with NDUFV1

  • NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa (Ndufv1)
  • NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa (NDUFV1)
  • NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa (ndufv1)
  • NADH dehydrogenase ubiquinone flavoprotein 1 (ndufv1)
  • NADH dehydrogenase (ubiquinone) flavoprotein 1 (ndufv1)
  • NADH dehydrogenase (ubiquinone) flavoprotein 1 (Ndufv1)
  • CI-51K protein
  • CI-51kD protein
  • CI51KD protein
  • DDBDRAFT_0188174 protein
  • DDBDRAFT_0191420 protein
  • DDB_0188174 protein
  • DDB_0191420 protein
  • GB17095 protein
  • UQOR1 protein
  • wu:fc01f01 protein
  • wu:fc12f12 protein
  • zgc:86620 protein

Protein level used designations for NDUFV1

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 , CI-51kD , NADH dehydrogenase flavoprotein 1 , NADH-ubiquinone oxidoreductase 51 kDa subunit, mitochondrial , mitochondrial complex I subunit NDUFV1

GENE ID SPECIES
408367 Apis mellifera
430210 Gallus gallus
496988 Xenopus (Silurana) tropicalis
8626872 Dictyostelium discoideum AX4
100122492 Nasonia vitripennis
100179486 Ciona intestinalis
445291 Danio rerio
17995 Mus musculus
293655 Rattus norvegicus
379907 Xenopus laevis
4723 Homo sapiens
476004 Canis lupus familiaris
287014 Bos taurus
747209 Pan troglodytes
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