Product info:

This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.

Alternative names:

BCKDHB; BCKDE1B; BCKDH E1-beta; E1B; BCKDHE1-beta

Species reactivity:

Mouse

Host:

Rabbit

Cellular localisation:

Mitochondrion matrix

Isotype:

IgG

Immunogen:

Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1).

Positive control:

Mouse pancreas

AA Sequence:

RSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY

Purification:

Affinity purification

Molecular weight:

Calculated MW: 23kDa/43kDa Observed MW: 43kDa

Form:

Liquid

Applications:

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Dilution:

WB 1:500 - 1:2000

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.

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