catalog number :
MBS9203560
products full name :
NDUFS7 Antibody (Center)
products short name :
[NDUFS7]
products name syn :
[NADH dehydrogenase [ubiquinone] iron-sulfur protein 7; mitochondrial; Complex I-20kD; CI-20kD; NADH-ubiquinone oxidoreductase 20 kDa subunit; PSST subunit; NDUFS7]
other names :
[NADH dehydrogenase; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial; NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase); Complex I-20kD; CI-20kD; NADH-ubiquinone oxidoreductase 20 kDa subunit; PSST subunit]
products gene name :
[NDUFS7]
other gene names :
[NDUFS7; NDUFS7; PSST; CI-20; MY017; CI-20KD; CI-20kD]
uniprot entry name :
NDUS7_HUMAN
reactivity :
Human, mouse (Predicted Reactivity: Bovine)
sequence positions :
[119-146]
specificity :
This NDUFS7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 119-146 amino acids from the Central region of human NDUFS7.
purity :
Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
form :
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 stability :
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.
tested application :
Western Blot (WB), ELISA (EIA)
image1 heading :
Western Blot (WB)
other info1 :
Calculated MW: 23564
other info2 :
Function: 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 (By similarity). Cellular Location: Mitochondrion.
products categories :
Crown Antibodies; Metabolism; Neuroscience; Signal Transduction
products description :
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 (By similarity).
products references :
Hyslop S.J., et al. Genomics 37:375-380(1996). Grimwood J., et al. Nature 428:529-535(2004). Murray J., et al. J. Biol. Chem. 278:13619-13622(2003). Burkard T.R., et al. BMC Syst. Biol. 5:17-17(2011). Triepels R.H., et al. Ann. Neurol. 45:787-790(1999).
ncbi acc num :
NP_077718.3
ncbi gb acc num :
NM_024407.4
ncbi summary :
This gene encodes a protein that is a subunit of one of the complexes that forms the mitochondrial respiratory chain. This protein is one of over 40 subunits found in complex I, the nicotinamide adenine dinucleotide (NADH):ubiquinone oxidoreductase. This complex functions in the transfer of electrons from NADH to the respiratory chain, and ubiquinone is believed to be the immediate electron acceptor for the enzyme. Mutations in this gene cause Leigh syndrome due to mitochondrial complex I deficiency, a severe neurological disorder that results in bilaterally symmetrical necrotic lesions in subcortical brain regions. [provided by RefSeq, Jul 2008]
uniprot summary :
NDUFS7: 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 NDUFS7 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 NDUFS7 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 20 kDa subunit family. Protein type: EC 1.6.99.3; Oxidoreductase; Mitochondrial; EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation. Chromosomal Location of Human Ortholog: 19p13.3. Cellular Component: neuron projection; cell soma; mitochondrial inner membrane; mitochondrial respiratory chain complex I. Molecular Function: oxidoreductase activity, acting on NADH or NADPH, quinone or similar compound as acceptor; protein binding; protease binding; NADH dehydrogenase (ubiquinone) activity; 4 iron, 4 sulfur cluster binding; metal ion binding; quinone binding; NADH dehydrogenase activity. Biological Process: cellular metabolic process; mitochondrial respiratory chain complex I assembly; mitochondrial electron transport, NADH to ubiquinone. Disease: Leigh Syndrome