NDUFV1 Antibody(N-term)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| WB, E |
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Primary Accession | P49821 |
Other Accession | Q91YT0, Q8HXQ9, P25708, NP_009034.2 |
Reactivity | Human |
Predicted | Bovine, Monkey, Mouse |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 50817 Da |
Antigen Region | 21-50 aa |
Gene ID | 4723 |
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Other Names | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial, Complex I-51kD, CI-51kD, NADH dehydrogenase flavoprotein 1, NADH-ubiquinone oxidoreductase 51 kDa subunit, NDUFV1, UQOR1 |
Target/Specificity | This NDUFV1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 21-50 amino acids from the N-terminal region of human NDUFV1. |
Dilution | WB~~1:1000 |
Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | NDUFV1 Antibody(N-term) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | NDUFV1 (HGNC:7716) |
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Synonyms | UQOR1 |
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:28844695). Part of the peripheral arm of the enzyme, where the electrons from NADH are accepted by flavin mononucleotide (FMN) and then passed along a chain of iron-sulfur clusters by electron tunnelling to the final acceptor ubiquinone (PubMed:28844695). Contains FMN, which is the initial electron acceptor as well as one iron-sulfur cluster (PubMed:28844695). |
Cellular Location | Mitochondrion inner membrane {ECO:0000250|UniProtKB:P25708}; Peripheral membrane protein {ECO:0000250|UniProtKB:P25708}; Matrix side {ECO:0000250|UniProtKB:P25708} |
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Background
The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
References
Wang, W., et al. Nucleic Acids Res. (2010) In press :
Moran, M., et al. Biochim. Biophys. Acta 1802(5):443-453(2010)
Saito, A., et al. J. Hum. Genet. 54(6):317-323(2009)
Starr, J.M., et al. Mech. Ageing Dev. 129(12):745-751(2008)
Ben-Shachar, D., et al. PLoS ONE 2 (9), E817 (2007) :
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