Target：NDUV1

Fields：Oxidative phosphorylation;Metabolic pathways;Thermogenesis;Retrograde endocannabinoid signaling;Non-alcoholic fatty liver disease;Alzheimer disease;Parkinson disease;Amyotrophic lateral sclerosis;Huntington disease;Prion disease;Pathways of neurodegeneration - multiple diseases;Chemical carcinogenesis - reactive oxygen species;Diabetic cardiomyopathy

Gene Name：NDUFV1 UQOR1

Protein Name：NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-51kD) (CI-51kD) (NADH dehydrogenase flavoprotein 1) (NADH-ubiquinone oxidoreductase 51 kDa subunit)

Human Gene Id：4723

Human Swiss Prot No：P49821

Mouse Swiss Prot No：Q91YT0

Immunogen：Synthesized peptide derived from human protein . at AA range: 170-250

Specificity：NDUV1 Polyclonal Antibody detects endogenous levels of protein.

Formulation：Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000 ELISA 1:5000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：51kD

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 trans

Function：catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,cofactor:Binds 1 4Fe-4S cluster .,cofactor:Binds 1 FMN .,disease:Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,disease:Defects in NDUFV1 are a cause of mitochondrial complex I deficiency [MIM:252010]. Complex I (NADH-ubiquinone oxidoreductase), the largest complex of the mitochondrial respiratory chain, contains more than 40 subunits. It is embedded in the inner mitochondrial membrane and is partly protruding in the matrix. Complex I deficiency is the most common cause of mitochondrial disorders. It represents largely one-third of all cases of respiratory chain deficiency and is responsible for a variety of clinical symptom

Subcellular Location：Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .

Expression： Brain,Eye,Kidney,Pituitary,