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

NDUFV1 (NADH Dehydrogenase [Ubiquinone] Flavoprotein 1, Mitochondrial, NADH-Ubiquinone Oxidoreductase 51kD Subunit, Complex I-51kD, CI-51kD, NADH Dehydrogenase Flavoprotein 1, UQOR1) (FITC)

Cat. Num. AAA6386768

• Gene Names: NDUFV1; UQOR1; CI-51K; CI51KD
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NDUFV1; Polyclonal Antibody; NDUFV1 (NADH Dehydrogenase [Ubiquinone] Flavoprotein 1; Mitochondrial; NADH-Ubiquinone Oxidoreductase 51kD Subunit; Complex I-51kD; CI-51kD; NADH Dehydrogenase Flavoprotein 1; UQOR1) (FITC); anti-NDUFV1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human NDUFV1.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-NDUFV1 antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human NDUFV1, aa1-464 (NP_009034.2).
• Immunogen Sequence: MLATRRLLGWSLPARVSVRFSGDTTAPKKTSFGSLKDEDRIFTNLYGRHDWRLKGSLSRGDWYKTKEILLKGPDWILGEIKTSGLRGRGGAGFPTGLKWSFMNKPSDGRPKYLVVNADEGEPGTCKDREILRHDPHKLLEGCLVGGRAMGARAAYIYIRGEFYNEASNLQVAIREAYEAGLIGKNACGSGYDFDVFVVRGAGAYICGEETALIESIEGKQGKPRLKPPFPADVGVFGCPTTVANVETVAVSPTICRRGGTWFAGFGRERNSGTKLFNISGHVNHPCTVEEEMSVPLKELIEKHAGGVTGGWDNLLAVIPGGSSTPLIPKSVCETVLMDFDALVQAQTGLGTAAVIVMDRSTDIVKAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWEISKQIEGHTICALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS
• Conjugate: FITC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of NDUFV1 expression in transfected 293T cell line by NDUFV1 polyclonal antibody. Lane 1: NDUFV1 transfected lysate (50.8kD). Lane 2: Non-transfected lysate. )

Product Categories/Family for anti-NDUFV1 antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 20149568
• NCBI GeneID: 4723
• NCBI Accession #: NP_009034.2
• NCBI GenBank Nucleotide #: NM_007103.2
• 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