BCKDHA Antibodies

AND BCKDHA ELISA kits, BCKDHA Proteins

Many biological assays use antibodies to detect BCKDHA, such as Western Blot (WB), ELISA, Flow Cytometry, Immunocytochemistry (IHC). These antibodies can be polyclonal or monoclonal, and react with BCKDHA in Human, Mouse, Rat, and many other animal samples. Boster Bio use mainly mouse and rabbit to develop BCKDHA antibodies...

We validate the specificity of these antibodies to BCKDHA by testing them on tissues known to express BCKDHA positively and negatively. Browse below to find the BCKDHA antibody that suites your experiment. We have 3 of these antibodies and many publications and validation images.

If you cannot find antibodies that fit your needs, contact us for making custom antibodies. We have a full suite of custom antibody services covering from research to diagnostic and therapeutic applications.

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BCKDHA Infographic by Boster Bio

All BCKDHA Products

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Product

Figures

Specificity

Applications

Price

Anti-BCKDHA Antibody

Human, Mouse, Rat

IF, ICC, WB

$405
Cat # A04561
100ul

Human BCKDHA Recombinant Protein

SDS-PAGE

$300
Cat # PROTP12694
Starting from 10ug

Anti-BCKDHA Antibody Picoband®

+11

Human, Mouse, Rat

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

$370
Cat # A04561-1
100 μg/vial

BCKDHA Overview

Facts about 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial.

BCKDHA 3D structure. Source: alphafold

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA)

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids into acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

Gene Information

Human
Gene Name:	BCKDHA
Uniprot:	P12694
Entrez:	593

Family

Belongs to:

BCKDHA family

Alternative Names

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urinedisease); branched chain keto acid dehydrogenase E1, alpha polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; EC 1.2.4.4,2-oxoisovalerate dehydrogenase (lipoamide); FLJ45695; MSU; MSUD1; OVD1A

Molecular Weight

Mass (kDA):
50.471 kDA

Genomic Context


Human

Location:	19q13.2
Sequence:	19; NC_000019.10 (41397818..41425002)

Subcellular Localizations

Mitochondrion matrix.

Diseases

Maple Syrup Urine Disease
Metabolic Diseases
Diabetes Mellitus
Lethargy
Adenoma
Malignant Hyperpyrexia Due To Anesthesia
Myopathy
Fibrosis
Neoplasms
Hirschsprung Disease
Cytogenetic Abnormality

Myotonic Dystrophy
Thyroid Gland Follicular Adenoma
Dysequilibrium Syndrome
Equine Infectious Anemia
Disorder Of Branched-chain Amino Acid Metabolism
Congenital Nephrotic Syndrome

Interacting Proteins

BCKDHB

Pathways

Hibernation
Flight
Urea Cycle
Reverse Transcription
Gluconeogenesis
Glycolysis
Localization

Embryo Development

PTMs

Acetylation

Phosphoprotein

For details, visit:
bosterbio.com/bosterbio-gene-info-cards/BCKDHA

References

PMID: 1420356 by McKean M.C., et al. Nucleotide sequence of the 5' end including the initiation codon of cDNA for the E1 alpha subunit of the human branched chain alpha- ketoacid dehydrogenase complex.

PMID: 2914958 by Fisher C.W., et al. Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex.