BCKDHB Monoclonal antibody, PBS Only (Detector)

BCKDHB Monoclonal Antibody for Cytometric bead array, Indirect ELISA

Host / Isotype

Mouse / IgG1

Reactivity

human, mouse, rat

Applications

Cytometric bead array, Indirect ELISA

Conjugate

Unconjugated

CloneNo.

1D12C7

Cat no : 68596-1-PBS

Synonyms

E1B, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDH E1-beta, BCKDE1B, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial



Product Information

68596-1-PBS targets BCKDHB as part of a matched antibody pair:

MP50570-1: 68596-2-PBS capture and 68596-1-PBS detection (validated in Cytometric bead array)

MP50570-3: 68596-4-PBS capture and 68596-1-PBS detection (validated in Cytometric bead array)

Unconjugated mouse monoclonal antibody pair in PBS only (BSA and azide free) storage buffer at a concentration of 1 mg/mL, ready for conjugation.

This conjugation ready format makes antibodies ideal for use in many applications including: ELISAs, multiplex assays requiring matched pairs, mass cytometry, and multiplex imaging applications.Antibody use should be optimized by the end user for each application and assay.

Tested Reactivity human, mouse, rat
Host / Isotype Mouse / IgG1
Class Monoclonal
Type Antibody
Immunogen BCKDHB fusion protein Ag33802
Full Name branched chain keto acid dehydrogenase E1, beta polypeptide
Calculated Molecular Weight 392 aa, 43 kDa
Observed Molecular Weight35-37 kDa
GenBank Accession NumberBC040139
Gene Symbol BCKDHB
Gene ID (NCBI) 594
Conjugate Unconjugated
Form Liquid
Purification MethodProtein G purification
Storage Buffer PBS Only
Storage ConditionsStore at -80°C.

Background Information

Branched-chain alpha-keto acid dehydrogenase E1 component beta chain (BCKDHB; BCKDE1B; BCKDH E1-beta) is a subunit of the BCKDH complex, which is a mitochondrial enzyme in the degradation pathway for branched-chain amino acids (BCAA). Together with BCKDHA, BCKDHB forms the E1 subunit of this complex, whereas DBT and DLD are the E2 and E3 subunits, respectively. A deficiency of the BCKDH complex in humans causes maple syrup urine disease (MSUD), a severe neurometabolic disorder diagnosed by the detection alloisoleucine in plasma (MIM 248600). (PMID: 30709776)