Anti-BCKDHA Antibody Picoband®

SKU: A04561-1

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

Application Details: Western blot, 0.1-0.25 μg/ml, Human, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: BCKDHA

Specificity:

Background: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene. The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.

Immunogen: E.coli-derived human BCKDHA recombinant protein (Position: A30-K445).

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. Chuang, J. L., Davie, J. R., Chinsky, J. M., Wynn, R. M., Cox, R. P., Chuang, D. T. Molecular and biochemical basis of intermediate maple syrup urine disease: occurrence of homozygous G245R and F364C mutations at the E1-alpha locus of Hispanic-Mexican patients. J. Clin. Invest. 95: 954-963, 1995. 2. Chuang, J. L., Fisher, C. R., Cox, R. P., Chuang, D. T. Molecular basis of maple syrup urine disease: novel mutations at the E1-alpha locus that impair E1(alpha-2-beta-2) assembly or decrease steady-state E1-alpha mRNA levels of branched-chain alpha-keto acid dehydrogenase complex. Am. J. Hum. Genet. 55: 297-304, 1994. 3. Chuang, J. L., Wynn, R. M., Moss, C. C., Song, J., Li, J., Awad, N., Mandel, H., Chuang, D. T. Structural and biochemical basis for novel mutations in homozygous Israeli maple syrup urine disease patients. J. Biol. Chem. 279: 17792-17800, 2004.

Uniprot ID: P12694

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: 50 kDa

Calculated Molecular Weight:

Gene ID: 593

Protein Name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Gene Full Name: branched chain keto acid dehydrogenase E1 subunit alpha

Synonyms: BCKDHA; FLJ45695; MSU; MSUD1; OVD1A