BCKDHA
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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)
Recombinant Proteins
- Human
- Zebrafish
- Cynomolgus
- Wheat Germ
- E.coli
- Mammalian Cell
- In Vitro Cell Free System
- E. coli
- HEK293
- GST
- His
- Non
- His&Fc&Avi
| Cat.# | Product name | Source (Host) | Species | Tag | Protein Length | Price |
|---|---|---|---|---|---|---|
| BCKDHA-135H | Recombinant Human BCKDHA Protein, GST-tagged | Wheat Germ | Human | GST |
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| BCKDHA-136H | Recombinant Human BCKDHA Protein, GST-tagged | Wheat Germ | Human | GST |
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| BCKDHA-27439TH | Recombinant Human BCKDHA, His-tagged | E.coli | Human | His | 51-445 a.a. |
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| BCKDHA-27442TH | Recombinant Human BCKDHA, His-tagged | E.coli | Human | His | 400 amino acids |
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| BCKDHA-3020Z | Recombinant Zebrafish BCKDHA | Mammalian Cell | Zebrafish | His |
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| BCKDHA-338C | Recombinant Cynomolgus BCKDHA Protein, His-tagged | Mammalian Cell | Cynomolgus | His |
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| BCKDHA-962H | Recombinant Human BCKDHA | E.coli | Human | His | 46-445 a.a. |
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| BCKDHA-163HCL | Recombinant Human BCKDHA cell lysate | Human | Non |
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| BCKDHA-1630HF | Recombinant Full Length Human BCKDHA Protein, GST-tagged | In Vitro Cell Free System | Human | GST | Full L. 445 amino acids |
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| BCKDHA-19H | Recombinant Human BCKDHA protein, His-tagged | E. coli | Human | His | 264-445 aa |
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| BCKDHA-86C | Recombinant Cynomolgus Monkey BCKDHA Protein, His (Fc)-Avi-tagged | HEK293 | Cynomolgus | His&Fc&Avi |
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| BCKDHA-86C-B | Recombinant Cynomolgus Monkey BCKDHA Protein Pre-coupled Magnetic Beads | HEK293 | Cynomolgus |
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Involved Pathway
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 | DLDH,ACAT1,AUH,HMGCLL1,MCCC1,ALDH3A2,ACAT2,ACAA2,ACADS,ALDH7A1 |
| Propanoate metabolism | HADHAB,BCKDHB,HIBCH,ACACA,PCCB,ACAT2,LDHBA,HADHAA,LDHBB,LDHA |
| Metabolic pathways | REV3L,POLD1,DNMT3AA,ACOX2,KCNG4,ALOX8,LIPCA,GLSA,ALG8,P4HA1A |
Protein Function
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 |
| alpha-ketoacid dehydrogenase activity | BCKDHB |
| metal ion binding | CCDC71L,MGRN1A,MMP13A,ZNF649,UQCRC2B,CYP46A1.1,NENF,MAP2K2,SP2,EPT1 |
| carboxy-lyase activity | BCKDHB,PDXDC1,ECHDC1,LOC650128,GAD1B,SGPL1,URAD,MVDA,AMD2,PDXDC2P |
| protein binding | FABP5,TBL1XR1,DARS2,CRYGD,CDA,TECR,CD84,OBFC1,PTPRR,PID1 |
Interacting Protein
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
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