Rabbit anti-Human NDUFV1 Polyclonal Antibody | anti-NDUFV1 antibody

NDUFV1, NT (NDUFV1, UQOR1, NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial, Complex I-51kD, NADH dehydrogenase flavoprotein 1, NADH-ubiquinone oxidoreductase 51kD subunit) (APC)

Cat. Num. AAA6324464

• Gene Names: NDUFV1; UQOR1; CI-51K; CI51KD
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NDUFV1; Polyclonal Antibody; NT (NDUFV1; UQOR1; NADH dehydrogenase [ubiquinone] flavoprotein 1; mitochondrial; Complex I-51kD; NADH dehydrogenase flavoprotein 1; NADH-ubiquinone oxidoreductase 51kD subunit) (APC); NADH-ubiquinone oxidoreductase 51kD subunit; anti-NDUFV1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-NDUFV1 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: NDUFV1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 20-50 amino acids from the N-terminal region of human NDUFV1.
• Conjugate: APC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(NDUFV1 Antibody (N-term) western blot analysis in MDA-MB453 cell line lysates (35ug/lane).This demonstrates the NDUFV1 antibody detected the NDUFV1 protein (arrow).)

Product Categories/Family for anti-NDUFV1 antibody

Antibodies; Mitochondrial Proteins

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).

NCBI and Uniprot Product Information

• NCBI GI #: 20149568
• NCBI GeneID: 4723
• NCBI Accession #: NP_009034.2
• NCBI GenBank Nucleotide #: NM_007103.3
• UniProt Accession #: P49821; O60924; O60940; Q16104; Q6IBR3; Q96BF8; Q96HS7
• Molecular Weight: 49,868 Da
• NCBI Official Full Name: NADH dehydrogenase
• NCBI Official Synonym Full Names: NADH:ubiquinone oxidoreductase core subunit V1
• NCBI Official Symbol: NDUFV1
• NCBI Official Synonym Symbols: UQOR1; CI-51K; CI51KD
• NCBI Protein Information: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
• UniProt Protein Name: NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
• UniProt Gene Name: NDUFV1
• UniProt Synonym Gene Names: UQOR1; CI-51kD
• UniProt Entry Name: NDUV1_HUMAN

NCBI Description

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.[provided by RefSeq, Oct 2009]

Uniprot Description

NDUFV1: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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. Defects in NDUFV1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, 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. Belongs to the complex I 51 kDa subunit family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.6.99.3; EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 11q13

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: NADH dehydrogenase (ubiquinone) activity; FMN binding; metal ion binding; 4 iron, 4 sulfur cluster binding; NAD binding

Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone

Disease: Mitochondrial Complex I Deficiency