NDUFB9

The protein encoded by this gene is a subunit of the mitochondrial oxidative phosphorylation complex I (nicotinamide adenine dinucleotide: ubiquinone oxidoreductase). Complex I is localized to the inner mitochondrial membrane and functions to dehydrogenate nicotinamide adenine dinucleotide and to shuttle electrons to coenzyme Q. Complex I deficiency is the most common defect found in oxidative phosphorylation disorders and results in a range of conditions, including lethal neonatal disease, hypertrophic cardiomyopathy, liver disease, and adult-onset neurodegenerative disorders. Pseudogenes of this gene are found on chromosomes five, seven and eight. Alternative splicing results in multiple transcript variants.
Full Name
NADH:Ubiquinone Oxidoreductase Subunit B9
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Biological Process
Aerobic respiration Source: ComplexPortal
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: ProtInc
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Sensory perception of sound Source: ProtInc
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 24 (MC1DN24):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. MC1DN24 transmission pattern is consistent with autosomal recessive inheritance.
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Anti-NDUFB9 antibodies

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Rabbit Anti-NDUFB9 Recombinant Antibody (CBWJN-1271) (CBMAB-N1671-WJ)

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Target: NDUFB9
Host: Rabbit
Antibody Isotype: IgG
Specificity: Mouse, Rat, Human
Clone: CBWJN-1271
Application*: WB, IP, P, F, IC
Datasheet SDS

Rabbit Anti-NDUFB9 Recombinant Antibody (CBWJN-1270) (CBMAB-N1670-WJ)

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Target: NDUFB9
Host: Rabbit
Antibody Isotype: IgG
Specificity: Rat, Human
Clone: CBWJN-1270
Application*: WB, IP, P, F, IC
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (D-7) (CBMAB-N1669-WJ)

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Target: NDUFB9
Host: Mouse
Specificity: Human
Clone: D-7
Application*: WB, IP, IF, E
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (9B2) (CBMAB-N1668-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 9B2
Application*: WB, F
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (8B7) (CBMAB-N1667-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Monkey
Clone: 8B7
Application*: WB, F
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (4F8) (CBMAB-N1666-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Rat, Mouse
Clone: 4F8
Application*: WB, IH
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (3A1) (CBMAB-N1665-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 3A1
Application*: WB, IH
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (CBWJN-0187) (CBMAB-N1664-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBWJN-0187
Application*: WB, IH, F
Datasheet SDS

Mouse Anti-NDUFB9 Recombinant Antibody (3E11) (CBMAB-N1663-WJ)

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Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: 3E11
Application*: WB
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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