catalog number :
MBS2517949
products full name :
NDUFS2 Polyclonal Antibody
products short name :
[NDUFS2]
products name syn :
[CI-49]
other names :
[NADH dehydrogenase; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit; NADH-ubiquinone oxidoreductase NDUFS2 subunit; complex 1, mitochondrial respiratory chain, 49-KD subunit; complex I 49kDa subunit; complex I-49kD; NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase); Complex I-49kD; CI-49kD; NADH-ubiquinone oxidoreductase 49 kDa subunit]
products gene name :
[NDUFS2]
other gene names :
[NDUFS2; NDUFS2; CI-49; CI-49kD]
uniprot entry name :
NDUS2_HUMAN
reactivity :
Human, Mouse, Rat
purity :
Affinity purification
storage stability :
Store at -20 degree C. Avoid freeze / thaw cycles.
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
app notes :
WB: 1:200-1:1000. IHC: 1:50-1:200
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: Synthetic peptide of human NDUFS2. Calculated Molecular Weight: 53kDa
other info2 :
Buffer: PBS with 0.05% sodium azide, 50% glycerol, pH7.3
products description :
The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
ncbi gb acc num :
NM_001166159.1
ncbi mol weight :
51,852 Da
ncbi pathways :
Alzheimer's Disease Pathway (83097); Alzheimer's Disease Pathway (509); Electron Transport Chain Pathway (198860); Huntington's Disease Pathway (83100); Huntington's Disease Pathway (512); Metabolic Pathways (132956); Metabolism Pathway (477135); NADH Dehydrogenase (ubiquinone) Fe-S Protein/flavoprotein Complex, Mitochondria Pathway (413407); NADH Dehydrogenase (ubiquinone) Fe-S Protein/flavoprotein Complex, Mitochondria Pathway (890441); Non-alcoholic Fatty Liver Disease (NAFLD) Pathway (862188)
ncbi summary :
The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]
uniprot summary :
NDUFS2: 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 NDUFS2 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 49 kDa subunit family. Protein type: Mitochondrial; EC 1.6.99.3; EC 1.6.5.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation. Chromosomal Location of Human Ortholog: 1q23. Cellular Component: nucleoplasm; mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I. Molecular Function: protein binding; electron carrier activity; NADH dehydrogenase (ubiquinone) activity; metal ion binding; 4 iron, 4 sulfur cluster binding; quinone binding; NAD binding; NADH dehydrogenase activity. Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone; response to oxidative stress. Disease: Mitochondrial Complex I Deficiency