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Mouse Anti-NDUFA2 Recombinant Antibody (6E7) (CBMAB-N1602-WJ)

This product is a Mouse antibody that recognizes NDUFA2. The antibody 6E7 can be used for immunoassay techniques such as: ELISA, WB.
See all NDUFA2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
6E7
Antibody Isotype
IgG1, κ
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1, 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 alpha subcomplex, 2, 8kDa
Introduction
The encoded protein is a subunit of the hydrophobic protein fraction of the NADH:ubiquinone oxidoreductase (complex 1), the first enzyme complex in the electron transport chain located in the inner mitochondrial membrane, and may be involved in regulating complex I activity or its assembly via assistance in redox processes. Mutations in this gene are associated with Leigh syndrome, an early-onset progressive neurodegenerative disorder. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]
Entrez Gene ID
4695
UniProt ID
O43678
Alternative Names
NADH:Ubiquinone Oxidoreductase Subunit A2; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex, 2, 8kDa; Complex I B8 Subunit; NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex, 2 (8kD, B8); NADH Dehydrogenase [Ubiquinone] 1 Alpha Subcomplex Subunit 2; NADH-Ubiquinone Oxidoreductase Subunit CI-B8; NADH-Ubiquinone Oxidoreductase B8 Subunit;
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
Blastocyst hatching Source: Ensembl
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 13 (MC1DN13):
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. MC1DN13 transmission pattern is consistent with autosomal recessive inheritance.
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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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