NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) Protéines (NDUFS4)

NDUFS4 encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), or NADH:ubiquinone oxidoreductase, the first multi-subunit enzyme complex of the mitochondrial respiratory chain. De plus, nous expédions NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) Anticorps (71) et beaucoup plus de produits pour cette protéine.

afficher tous les protéines Gène GeneID UniProt
NDUFS4 17993 Q9CXZ1
Rat NDUFS4 NDUFS4 499529 Q5XIF3
NDUFS4 4724 O43181
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Escherichia coli (E. coli) Souris His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Connectez-vous pour afficher 30 to 35 Days
$5,370.21
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Escherichia coli (E. coli) Humain His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Connectez-vous pour afficher 30 to 35 Days
$5,370.21
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Wheat germ Humain GST tag 10 μg Connectez-vous pour afficher 11 to 12 Days
$340.00
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HEK-293 Cells Humain Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Connectez-vous pour afficher 11 Days
$888.80
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Levure Pongo pygmaeus His tag   1 mg Connectez-vous pour afficher 60 to 71 Days
$2,277.00
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Levure Orang-Utan His tag   1 mg Connectez-vous pour afficher 60 to 71 Days
$2,277.00
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Levure Boeuf (Vache) His tag   1 mg Connectez-vous pour afficher 60 to 71 Days
$2,277.00
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Levure Chimpanzé His tag   1 mg Connectez-vous pour afficher 60 to 71 Days
$2,277.00
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Escherichia coli (E. coli) Humain Inconjugué 100 μg Connectez-vous pour afficher 15 to 19 Days
$400.00
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Escherichia coli (E. coli) Humain His tag   1 mg Connectez-vous pour afficher 2 to 3 Days
$3,655.38
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NDUFS4 Protéines protéines par origine et source

Origin Exprimée danse Conjugué
Mouse (Murine)

Human , ,
, ,

Plus protéines pour NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) (NDUFS4) partenaires d'interaction

Mouse (Murine) NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) (NDUFS4) interaction partners

  1. the partial absence of complex I sensitizes the myocardium towards ischemia reperfusion injury and that the main source of reactive oxygen species following reperfusion is complex III.

  2. Excitatory synaptic transmission in the parietal association cortex in slices from Ndufs4(KO) animals was hypersensitive to isoflurane compared to control slices. We identified a direct neural circuit between the parietal association cortex and the central thalamus, consistent with a model in which isoflurane sensitivity is mediated by a thalamic signal relayed through excitatory synapses to the parietal association cort

  3. Study showed that the earliest cell loss in complex 1-deficient Ndufs4 mice retinas is retinal bipolar cells at p20, followed by Starburst Amacrine Cells at p24, that precede a rise in inflammatory molecules at p30 and retinal ganglion cell death at p42; results could suggest a mechanism in which the death of bipolar and amacrine cells incite an inflammatory wave that ultimately results in retinal ganglion cell loss.

  4. NDUFS4 deletion affected gene expression following neural differentiation and the potential of the cells to generate beating embryoid bodies.

  5. Using the Ndufs4 knockout (Ndufs4 KO) mouse, a model of Leigh syndrome, we demonstrate for the first time that protein succination is increased in the brainstem (BS), particularly in the vestibular nucleus.

  6. Locally insufficient respiration capacity of the nerve terminals may drive focal neurodegeneration in the Ndufs4 knockout mouse model of the Leigh syndrome.

  7. This study demonstrated that Genetic reduction of Ndufs4 function does not lead to loss of dopamine neurons in vivo.

  8. Reduced adolescent-age spatial learning ability is associated with elevated juvenile-age superoxide levels in complex I mouse mutants.

  9. Mitochondrial complex I dysfunction in the retina, present in Ndufs4 KO mouse, triggers an innate immune and inflammatory response that results in loss of retinal ganglion cell function and death.

  10. Global Ndufs4 deletion causes systemic inflammation and osteopetrosis.

  11. Complex I deficiency due to selective loss of Ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy.

  12. deletion of Ndufs4 results in a significant loss of complex I-supported respiration in the heart, which is well tolerated with no major changes of cardiac function, energetics, and longevity of the mice under unstressed conditions.

  13. Oxidation of the reactive oxygen species sensor hydroethidium was increased and mitochondria were less branched and/or shorter in NDUFS4(-/-) fibroblasts.

  14. the NDUFS4 subunit is of key importance in CI stabilization

  15. results demonstrate that the loss of Ndufs4 causes only a mild complex I deficiency in vivo, which in dopamine neurons nevertheless leads to alterations that may play a causative or contributing role in the pathophysiology of late onset Parkinson's disease

  16. Proteomic 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) gene.

  17. Tthe absence of NDUFS4 in different mouse tissues results in decreased activity and stability of mitochondrial complex I.

  18. To explore the lethal, ataxic phenotype of complex I deficiency in Ndufs4 knockout mice, we inactivated Ndufs4 selectively in neurons and glia, resulting in progressive encephalopathy resembling Leigh syndrome.

  19. CI fails to assemble properly or is unstable without NDUFS4

  20. In this study, Ndufs4-/- mice was used as a genetic model to study mitochondrial complex I deficiency and dopaminergic neuron death.

Human NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) (NDUFS4) interaction partners

  1. The clinical presentations of five individuals of Hutterite descent with Leigh disease are described herein. An identity-by-descent mapping and candidate gene approach was used to identify a novel homozygous c.393dupA frameshift mutation in the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (NDUFS4) gene.

  2. The authors concluded that NDUFS4-related Leigh syndrome is invariably linked to an early onset severe phenotype that results in early death.

  3. The c.462delA deletion led to a complete lack of NDUFS4 peptide in isolated mitochondria, and this deficiency caused an inefficient mitochondrial complex I assembly and Leigh syndrome symptoms.

  4. Mutations in the NDUFS4 gene and its subunits are associated with the mitochondrial complex I deficiency. (Review)

  5. Elevated expression of CO I and ND4 were associated with gastric tumorigenesis and tumor dedifferentiation

  6. Studies indicate that that the functional capacity of complex I depends on phosphorylation and import of subunit NDUFS4 protein.

  7. In fibroblast cultures, protein kinase A-mediated phosphorylation of the NDUFS4 subunit of complex I rescues the activity of the oxidatively damaged complex.

  8. case Report: A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family.

  9. REVIEW: Phosphorylation of the NDUFS4 protein, overall respiratory activity, and mutations sassociated with deficiency of complex I.

  10. In patients with complex I deficiency, the increased whole-body oxygen consumption rate at rest reflects increased electron transport through the respiratory chain, driven by a decreased phosphorylation potential.

  11. observations show the essential role of the 18-kDa subunit of respiratory complex I (NDUFS4) gene in the structure and function of complex I and give insight into pathogenic mechanism of NDUFS4 gene mutations in a severe defect of complex I

  12. A nonsense mutation leading to the abrogation of mRNA decay was found in NDUFS4 gene of a Leigh syndrome patient.

  13. mutations in the NDUFS1 and NDUFS4 genes of complex I cause dysfunction in cellular oxidative metabolism

  14. NDUSF4 is required for the assembly and stabilization of a portion of complex I that contains a number of subunits.

  15. impact of PKA mediated phosphorylation on the mitochondrial import of in vitro and in vivo synthesized NDUFS4 protein

  16. regulation of alternative transcripts of the NDUFS4 gene of complex I of the respiratory chain

  17. NDUFS4 presents a hotspot of mutations in the genetic apparatus of oxidative phosphorylation and the correct assembly of the subunit it encodes is essential for completion of the assembly of complex I.

Cow (Bovine) NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) (NDUFS4) interaction partners

  1. Two-dimensional gel electrophoresis, (32)P labelling and immunodetection show that "in vitro" PKA phosphorylates the serine in the C-terminus of the NDUFS4 subunit in isolated bovine complex I.

Profil protéine NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4, 18kDa (NADH-Coenzyme Q Reductase) (NDUFS4)

Profil protéine

This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), or NADH:ubiquinone oxidoreductase, the first multi-subunit enzyme complex of the mitochondrial respiratory chain. Complex I plays a vital role in cellular ATP production, the primary source of energy for many crucial processes in living cells. It removes electrons from NADH and passes them by a series of different protein-coupled redox centers to the electron acceptor ubiquinone. In well-coupled mitochondria, the electron flux leads to ATP generation via the building of a proton gradient across the inner membrane. Complex I is composed of at least 41 subunits, of which 7 are encoded by the mitochondrial genome and the remainder by nuclear genes.

Gene names and symbols associated with NDUFS4

  • NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4)
  • NADH:ubiquinone oxidoreductase subunit S4 (Ndufs4)
  • NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4)
  • 6720411N02Rik Protéine
  • AQDQ Protéine
  • C1-18k Protéine
  • CI-18 Protéine

Protein level used designations for NDUFS4

CI-18 kDa , CI-AQDQ , NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial , NADH-ubiquinone oxidoreductase 18 kDa subunit , complex I-18 kDa , complex I-AQDQ , NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase) , NADH dehydrogenase (ubiquinone) iron-sulfur protein 4 , NADH-coenzyme Q reductase, 18-KD , complex I 18kDa subunit , mitochondrial respiratory chain complex I (18-KD subunit) , NADH-ubiquinone oxidoreductase 18 kDa subunit, mitochondrial

GENE ID SPECIES
17993 Mus musculus
499529 Rattus norvegicus
4724 Homo sapiens
374122 Gallus gallus
327680 Bos taurus
461878 Pan troglodytes
100172316 Pongo abelii
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