Molecular weight (DA)
23kD
Immunogen
Synthesized peptide derived from human protein . at AA range: 90-170
Specificity
NDUS8 Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome. [provided by RefSeq, Mar 2010],
Function
catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,cofactor:Binds 2 4Fe-4S clusters per subunit.,disease:Defects in NDUFS8 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.,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). May donate electrons to ubiquinone.,similarity:Belongs to the complex I 23 kDa subunit family.,similarity:Contains 2 4Fe-4S ferredoxin-type domains.,subunit:Mammalian complex I
Protein name
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-23kD) (CI-23kD) (NADH-ubiquinone oxidoreductase 23 kDa subunit) (TYKY subunit)
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
Human protein sequence Database
O00217
Mouse protein sequence database
Q8K3J1
Rat protein sequence database
Cellular localization
Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .
Tissue expression
Expressed in all tissues with the highest level in heart and skeletal muscle and the lowest level in lung.
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