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Mouse Anti-NDUFS8 Recombinant Antibody (A-6) (CBMAB-N1696-WJ)

This product is a Mouse antibody that recognizes NDUFS8. The antibody A-6 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all NDUFS8 antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
A-6
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
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!]

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:UBIQUINONE OXIDOREDUCTASE CORE SUBUNIT S8
Introduction
This gene encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome. [provided by RefSeq, Mar 2010]
Entrez Gene ID
Human4728
Mouse225887
Rat293652
UniProt ID
HumanO00217
MouseQ8K3J1
RatB0BNE6
Alternative Names
NADH:Ubiquinone Oxidoreductase Core Subunit S8; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 8, 23kDa (NADH-Coenzyme Q Reductase); NADH Dehydrogenase [Ubiquinone] Iron-Sulfur Protein 8, Mitochondrial; NADH-Ubiquinone Oxidoreductase 23 KDa Subunit; Complex I 23kDa Subunit; Complex I-23kD; NADH Dehydrogenase (Ubiquinone) Fe-S Protein 8 (23kD) (NADH-Coenzyme Q Reductase); TYKY Subunit;
Function
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:22499348).

Essential for the catalytic activity and assembly of complex I (PubMed:22499348).
Biological Process
Aerobic respiration Source: GO_Central
Mitochondrial ATP synthesis coupled proton transport Source: ComplexPortal
Mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Response to oxidative stress Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mitochondrial complex I deficiency, nuclear type 2 (MC1DN2):
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. MC1DN2 inheritance is autosomal recessive.
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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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