Anti-NDUFV2 Antibody Picoband®

SKU: A05801-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: ELISA,Flow Cytometry,IF,IHC,WB

Application Details: Western blot, 0.1-0.25 μg/ml/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml/ml, Human
Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/ml/1x10^6 cells, Human
ELISA, 0.1-0.5 μg/ml/ml, Human

Gene Name: NDUFV2

Specificity:

Background: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (NDUFV2) is an enzyme that in humans is encoded by the NDUFV2 gene. The NADH-ubiquinone oxidoreductase complex (complex I) of the mitochondrial respiratory chain catalyzes the transfer of electrons from NADH to ubiquinone, and consists of at least 43 subunits. The complex is located in the inner mitochondrial membrane. This gene encodes the 24 kDa subunit of complex I, and is involved in electron transfer. Mutations in this gene are implicated in Parkinson's disease, bipolar disorder, schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. A non-transcribed pseudogene of this locus is found on chromosome 19.

Immunogen: E.coli-derived human NDUFV2 recombinant protein (Position: M1-L249).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Benit, P., Beugnot, R., Chretien, D., Giurgea, I., De Lonlay-Debeney, P., Issartel, J.-P., Corral-Debrinski, M., Kerscher, S., Rustin, P., Rotig, A., Munnich, A. Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy. Hum. Mutat. 21: 582-586, 2003. 2. Cameron, J. M., MacKay, N., Feigenbaum, A., Tarnopolsky, M., Blaser, S., Robinson, B. H., Schulze, A. Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome. Europ. J. Paediat. Neurol. 19: 525-532, 2015. 3. de Coo, R., Buddiger, P., Smeets, H., Geurts van Kessel, A., Morgan-Hughes, J., Weghuis, D. O., Overhauser, J., van Oost, B. Molecular cloning and characterization of the active human mitochondrial NADH:ubiquinone oxidoreductase 24-kDa gene (NDUFV2) and its pseudogene. Genomics 26: 461-466, 1995.

Uniprot ID: P19404

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 27 kDa

Calculated Molecular Weight: 16693 MW

Gene ID: 4729

Protein Name: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial

Gene Full Name: NADH:ubiquinone oxidoreductase core subunit V2

Synonyms: NDUFV2