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
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.
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
+ Filters
Target: NDUFB9
Host: Rabbit
Antibody Isotype: IgG
Specificity: Mouse, Rat, Human
Clone: CBWJN-1271
Application*: WB, IP, P, F, IC
Target: NDUFB9
Host: Rabbit
Antibody Isotype: IgG
Specificity: Rat, Human
Clone: CBWJN-1270
Application*: WB, IP, P, F, IC
Target: NDUFB9
Host: Mouse
Specificity: Human
Clone: D-7
Application*: WB, IP, IF, E
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 9B2
Application*: WB, F
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Monkey
Clone: 8B7
Application*: WB, F
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Rat, Mouse
Clone: 4F8
Application*: WB, IH
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 3A1
Application*: WB, IH
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBWJN-0187
Application*: WB, IH, F
Target: NDUFB9
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: 3E11
Application*: WB
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For Research Use Only. Not For Clinical Use.
(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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