NDUFS7
Reactivité: Humain, Souris, Rat
ELISA, IHC
Hôte: Lapin
Polyclonal
unconjugated
Indications d'application
Peptide ELISA: Limit Dilution: 1/16000. Western blot: 1-3 μg/mL. This antibody detects a band of approx. 20 kDa in Human Heart andHuman Skeletal Muscle lysates. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions
For Research Use only
Concentration
0.5 mg/mL
Buffer
Tris saline, pH 7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer.
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Conseil sur la manipulation
Avoid repeated freezing and thawing.
Stock
4 °C/-20 °C
Stockage commentaire
Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Defects in NDUFS7 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. Defects in NDUFS7 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. 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.Synonyms: CI-20kD, Complex I-20kD, NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, NADH-ubiquinone oxidoreductase 20 kDa subunit, PSST subunit, mitochondrial