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Mouse Anti-NDUFB3 Recombinant Antibody (6C6) (CBMAB-N1651-WJ)

This product is a Mouse antibody that recognizes NDUFB3. The antibody 6C6 can be used for immunoassay techniques such as: ELISA, WB.
See all NDUFB3 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
6C6
Antibody Isotype
IgG2a, κ
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa
Introduction
This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which is the first enzyme in the electron transport chain of mitochondria. This protein localizes to the inner membrane of the mitochondrion as a single-pass membrane protein. Mutations in this gene contribute to mitochondrial complex 1 deficiency. Alternative splicing results in multiple transcript variants encoding the same protein. Humans have multiple pseudogenes of this gene. [provided by RefSeq, Mar 2012]
Entrez Gene ID
4709
UniProt ID
O43676
Alternative Names
NADH:Ubiquinone Oxidoreductase Subunit B3; NADH Dehydrogenase (Ubiquinone) 1 Beta Subcomplex, 3, 12kDa; NADH-Ubiquinone Oxidoreductase B12 Subunit; Complex I-B12; CI-B12; NADH Dehydrogenase (Ubiquinone) 1 Beta Subcomplex, 3 (12kD, B12); NADH Dehydrogenase [Ubiquinone] 1 Beta Subcomplex Subunit 3; Complex I B12 Subunit; B12;
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be 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
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 25 (MC1DN25):
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. MC1DN25 transmission pattern is consistent with autosomal recessive inheritance.
Topology
Helical: 66-88
PTM
Methylation at His residues by METTL9 enhances complex I-mediated mitochondrial respiration.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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