支链α-酮酸脱氢酶E2抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 支链α-酮酸脱氢酶E2抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

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,支链α-酮酸脱氢酶E2抗体 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. [provided by RefSeq, Jul 2008]

支链α-酮酸脱氢酶E2抗体Also known as:

BCATE2; BCKAD E2 subunit; Branched chain acyltransferase, E2 component; Dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex; maple syrup urine disease); Dihydrolipoamide branched chain transacylase; Dihydrolipoamide branched chain transacylase E2; Dihydrolipoyl transacylase; Dihydrolipoyllysine residue支链α-酮酸脱氢酶E2抗体 (2 methylpropanoyl)transferase; E2; ODB2_HUMAN; E2 component of branched chain alpha keto acid dehydrogenase complex; E2B; Lipoamide acyltransferase component of mitochondrial branched chain alpha keto acid dehydrogenase complex; Mitochond

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human DBT.

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支链α-酮酸脱氢酶E2抗体Predicted Molecular Weight: 46kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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