BCKDHB Monoklonaler Antikörper

BCKDHB Monoklonal Antikörper für Cytometric bead array, Indirect ELISA

Wirt / Isotyp

Maus / IgG1

Getestete Reaktivität

human, Maus, Ratte

Anwendung

Cytometric bead array, Indirect ELISA

Konjugation

Unkonjugiert

CloneNo.

1D12C7

Kat-Nr. : 68596-1-PBS

Synonyme

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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  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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Geprüfte Anwendungen

Produktinformation

68596-1-PBS bindet in Cytometric bead array, Indirect ELISA BCKDHB und zeigt Reaktivität mit human, Maus, Ratten

• Getestete Reaktivität: human, Maus, Ratte
• Wirt / Isotyp: Maus / IgG1
• Klonalität: Monoklonal
• Typ: Antikörper
• Immunogen: BCKDHB fusion protein Ag33802
• Vollständiger Name: branched chain keto acid dehydrogenase E1, beta polypeptide
• Berechnetes Molekulargewicht: 392 aa, 43 kDa
• Beobachtetes Molekulargewicht: 35-37 kDa
• GenBank-Zugangsnummer: BC040139
• Gene symbol: BCKDHB
• Gene ID (NCBI): 594
• Konjugation: Unkonjugiert
• Form: Liquid
• Reinigungsmethode: Protein-G-Reinigung
• Lagerungspuffer: PBS only
• Lagerungsbedingungen: Store at -80°C. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

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)