Anti-BCKDHA Antibody Picoband®

About this product

Boster Bio Anti-BCKDHA Antibody Picoband® catalog # A04561-1. Tested in ELISA, Flow Cytometry, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Specifications

Catalog number

A04561-1

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

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.

Synonyms

Lymphotactin; C motif chemokine 1; Cytokine SCM-1; Lymphotaxin; Small-inducible cytokine C1; Xcl1; Lptn; Ltn; Scyc1

Gene Name

BCKDHA

Gene ID

593

UniProt

P12694

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

Expressed in activated CD8 (+) T cells. In the thymus, expressed by medullary thymic epithelial cells.

Applications

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

Field of Research

Chemokines, Immunology, Innate Immunity

Purification

Immunogen affinity purified.

Concentration

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

Form

Lyophilized

Reconstitution

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

Function

Chemotactic activity for lymphocytes but not for monocytes or neutrophils. In thymus, mediates medullary accumulation of thymic dendritic cells and contributes to regulatoy T cell development, playing a role in self-tolerance establishment.

References & Citations

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.

Storage Conditions

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

Observed Molecular Weight

50 kDa

Applications Notes

6

Gene Name Synonym

Chemokine (C motif) ligand 1

Subcellular Location

Secreted.

Isotype

Rabbit IgG

Contents

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