Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA)

Cat.No	ACP24320	Target Name	BCKDHA
Target Synonyms	alpha polypeptide (maple syrup urine disease); Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; MSU; MSUD1; ODBA_HUMAN, Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide; FLJ45695; OVD1A; 2 oxoisovalerate dehydrogenase (lipoamide); 2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial ; 2-oxoisovalerate dehydrogenase subunit alpha	Form	Lyophilized powder
Expression System	Custom Production. Please inquire and provide the desire expression system.	Expression Range	46-445
Protein Length	Full Length of Mature Protein	Purity	>85% (SDS-PAGE)
Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Cat.No

ACP24320

Target Name

BCKDHA

Target Synonyms

alpha polypeptide (maple syrup urine disease); Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; MSU; MSUD1; ODBA_HUMAN, Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide; FLJ45695; OVD1A; 2 oxoisovalerate dehydrogenase (lipoamide); 2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial ; 2-oxoisovalerate dehydrogenase subunit alpha

Form

Lyophilized powder

Expression System

Custom Production. Please inquire and provide the desire expression system.

Expression Range

46-445

Protein Length

Full Length of Mature Protein

Purity

>85% (SDS-PAGE)

Storage Buffer

5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Target Species	Human	Uniprot ID	P12694

Background Information

Uniprot Id

P12694
Target Species

Human
Target Name

BCKDHA
Target Full Name

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Target Function

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Target Involvement

Maple syrup urine disease 1A (MSUD1A)
Target Subcellular Location

Mitochondrion matrix.
Target Protein Families

BCKDHA family
Target Synonyms

Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide; FLJ45695; OVD1A; 2 oxoisovalerate dehydrogenase (lipoamide); 2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial ; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1 alpha; BCKDH E1-alpha; BCKDHA; Branched chain alpha keto acid dehydrogenase E1 component alpha chain; Branched chain keto acid dehydrogenase E1 alpha polypeptide; Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease); Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; MSU; MSUD1; ODBA_HUMAN
Target Background

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.

Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA)

Specifications

Immunogen Information

Inquire Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA) Now

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