BCKDHB Antibodies

AND BCKDHB ELISA kits, BCKDHB Proteins

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

We validate the specificity of these antibodies to BCKDHB by testing them on tissues known to express BCKDHB positively and negatively. Browse below to find the BCKDHB antibody that suites your experiment. We have 1 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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BCKDHB Infographic by Boster Bio

All BCKDHB Products

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BCKDHB (NM_183050) Human Over-expression Lysate

Human

$628
Cat # LS000594
100 µg

BCKDHB Overview

Facts about 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial.

BCKDHB 3D structure. Source: alphafold

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BCKDHB)

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to 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:	BCKDHB
Uniprot:	P21953
Entrez:	594

Family

Belongs to:

No superfamily

Alternative Names

2-oxoisovalerate dehydrogenase beta subunit; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKDE1B; BCKDH E1-beta; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1, beta polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1B; E1b-beta subunit of the branched-chain complex; EC 1.2.4.4; FLJ17880

Molecular Weight

Mass (kDA):
43.122 kDA

Genomic Context


Human

Location:	6q14.1
Sequence:	6; NC_000006.12 (80106610..80469088)

Subcellular Localizations

Mitochondrion matrix.

Diseases

Maple Syrup Urine Disease
Metabolic Diseases
Disorder Of Branched-chain Amino Acid Metabolism
Profound Mental Retardation
Premature Menopause
Protein Truncation Abnormality
Equine Infectious Anemia

Turner Syndrome
Ovarian Failure, Premature

Interacting Proteins

BCKDHA

Pathways

Localization

PTMs

Acetylation

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

References

PMID: 2365818 by Matsuda I., et al. Maple syrup urine disease. Complete primary structure of the E1 beta subunit of human branched chain alpha-ketoacid dehydrogenase complex deduced from the nucleotide sequence and a gene analysis of patients with this disease.

PMID: 8651316 by Chuang J.L., et al. Maple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E1beta mRNAs arises from intronic sequences.