Product info:

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.

Alternative names:

E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCKDH-E2; BCOADC-E2

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

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Isotype:

IgG

Immunogen:

Recombinant protein of human DBT.

Positive control:

293T, HepG2, A375, Mouse lung, Mouse brain, Rat liver, Rat brain

AA Sequence:

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Purification:

Affinity purification

Molecular weight:

Calculated MW: 53kDa Observed MW: 53KDa

Form:

Liquid

Applications:

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Dilution:

WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

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Orders:

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