Anticorps Monoclonal anti-BCKDHB

BCKDHB Monoclonal Antibody for Cytometric bead array, Indirect ELISA

Hôte / Isotype

Mouse / IgG1

Réactivité testée

Humain, rat, souris

Applications

Cytometric bead array, Indirect ELISA

Conjugaison

Non conjugué

CloneNo.

1D12C7

N° de cat : 68596-1-PBS

Synonymes

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

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Galerie de données de validation

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  Cytometric bead array standard curve of MP50570-1

  Cytometric bead array standard curve of MP50570-1, BCKDHB Monoclonal Matched Antibody Pair, PBS Only. Capture antibody: 68596-2-PBS. Detection antibody: 68596-1-PBS. Standard:Ag33802. Range: 0.391-100 ng/mL.

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  Cytometric bead array standard curve of MP50570-3

  Cytometric bead array standard curve of MP50570-3, BCKDHB Monoclonal Matched Antibody Pair, PBS Only. Capture antibody: 68596-4-PBS. Detection antibody: 68596-1-PBS. Standard:Ag33802. Range: 1.563-100 ng/mL.

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Informations sur le produit

68596-1-PBS cible BCKDHB dans les applications de Cytometric bead array, Indirect ELISA et montre une réactivité avec des échantillons Humain, rat, souris

• Réactivité: Humain, rat, souris
• Hôte / Isotype: Mouse / IgG1
• Clonalité: Monoclonal
• Type: Anticorps
• Immunogène: BCKDHB Protéine recombinante Ag33802
• Nom complet: branched chain keto acid dehydrogenase E1, beta polypeptide
• Masse moléculaire calculée: 392 aa, 43 kDa
• Poids moléculaire observé: 35-37 kDa
• Numéro d’acquisition GenBank: BC040139
• Symbole du gène: BCKDHB
• Identification du gène (NCBI): 594
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par protéine G
• Tampon de stockage: PBS only
• Conditions de stockage: Store at -80°C. 20ul contiennent 0,1% de BSA.

Informations générales

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)

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