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BCKDHA

Official Full Name

branched chain keto acid dehydrogenase E1, alpha polypeptide
Overview

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.
Synonyms

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

Type

Recombinant Proteins
Cell & Tissue Lysates
Protein Pre-coupled Magnetic Beads

Species

Cynomolgus Monkey
Human
Zebrafish

Source

E.coli
HEK293
In Vitro Cell Free System
Mammalian Cell
Wheat Germ

Tag

GST
His
His (Fc)
Avi
N/A

Species	Cat.#	Product name	Source (Host)	Tag	Protein Length	Price
Human	BCKDHA-962H	Recombinant Human BCKDHA, His-tagged	E.coli	His		Inquiry
Human	BCKDHA-135H	Recombinant Human BCKDHA Protein, GST-tagged	Wheat Germ	GST		Inquiry
Human	BCKDHA-27439TH	Recombinant Human BCKDHA, His-tagged	E.coli	His		Inquiry
Human	BCKDHA-27442TH	Recombinant Human BCKDHA, His-tagged	E.coli	His	400 amino acids	Inquiry
Human	BCKDHA-136H	Recombinant Human BCKDHA Protein, GST-tagged	Wheat Germ	GST		Inquiry
Human	BCKDHA-163HCL	Recombinant Human BCKDHA cell lysate		N/A		Inquiry
Human	BCKDHA-1630HF	Recombinant Full Length Human BCKDHA Protein, GST-tagged	In Vitro Cell Free System	GST	445 amino acids	Inquiry
Cynomolgus Monkey	BCKDHA-338C	Recombinant Cynomolgus BCKDHA Protein, His-tagged	Mammalian Cell	His		Inquiry
Cynomolgus Monkey	BCKDHA-86C-B	Recombinant Cynomolgus Monkey BCKDHA Protein Pre-coupled Magnetic Beads	HEK293			Inquiry
Cynomolgus Monkey	BCKDHA-86C	Recombinant Cynomolgus Monkey BCKDHA Protein, His (Fc)-Avi-tagged	HEK293	His (Fc)-Avi		Inquiry
Zebrafish	BCKDHA-3020Z	Recombinant Zebrafish BCKDHA	Mammalian Cell	His		Inquiry

Involved Pathway
Protein Function
Interacting Protein

BCKDHA involved in several pathways and played different roles in them. We selected most pathways BCKDHA participated on our site, such as Valine, leucine and isoleucine degradation, Propanoate metabolism, Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with BCKDHA were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name	Pathway Related Protein
Valine, leucine and isoleucine degradation	ACAA1;MCCC1;MUT;AOX4;PCCA;AOX3L1;HADHAB;AOX1;PCCB
Propanoate metabolism	PCCA;ACSS2;HIBCH;LDHBB;MUT;MCEE;LDHC;MLYCD;ECHDC1
Metabolic pathways	LDHB;DDOST;AGL;ATP6V0B;PI4KB;ADSL;PIGC;AOC3;NDUFB6

BCKDHA has several biochemical functions, for example, 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity, alpha-ketoacid dehydrogenase activity, carboxy-lyase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by BCKDHA itself. We selected most functions BCKDHA had, and list some proteins which have the same functions with BCKDHA. You can find most of the proteins on our site.

Function	Related Protein
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity	BCKDHB;BCKDHA
alpha-ketoacid dehydrogenase activity	BCKDHB;BCKDHA
carboxy-lyase activity	BCKDHA;AMD2;PDXDC1;PDXDC2P;URAD;SGPL1;ECHDC1;LOC650128;GAD1B
metal ion binding	ALKBH7;CXXC1B;ABO;DBR1;FTR82;PPM1F;CTDSP2;ZBED4;HIVEP2
protein binding	CXorf40A;TFF2;TSSC4;COG1;NUMBL;DCTN1;CREB3L3;GZMB;NCBP1

BCKDHA has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with BCKDHA here. Most of them are supplied by our site. Hope this information will be useful for your research of BCKDHA.

BCKDHB; BCKDK; GABARAP; Pdlim5; GABARAPL1; HDAC6; SIRT4