BCKDHB
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Official Full Name
branched chain keto acid dehydrogenase E1, beta polypeptide -
Overview
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein 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 transcript variants with different 3 non-coding regions, but encoding the same isoform. -
Synonyms
BCKDHB;branched chain keto acid dehydrogenase E1, beta polypeptide;2-oxoisovalerate dehydrogenase subunit beta, mitochondrial;maple syrup urine disease;BCKDE1B;BCKDH E1-beta;2-oxoisovalerate dehydrogenase beta subunit;E1b-beta subunit of the branch
Recombinant Proteins
- Human
- Mouse
- Zebrafish
- Chicken
- Rat
- E.coli
- Wheat Germ
- Mammalian Cells
- HEK293
- In Vitro Cell Free System
- GST
- His
- Non
- Flag
- His&Fc&Avi
Involved Pathway
BCKDHB involved in several pathways and played different roles in them. We selected most pathways BCKDHB 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 BCKDHB were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Propanoate metabolism | ACACB,SUCLG2,ACSS2,ACADM,HIBCH,ACSS2L,LDHBA,ackA,SUCLG1,HADHAB |
| Metabolic pathways | HSD3B4,CMPK2,AMPD3B,PANK4,PTGES2,OXSR1,SPTLC2,GCH2,GPIB,SDHC |
| Valine, leucine and isoleucine degradation | ALDH3A2A,HMGCL,OXCT2,ACAA1A,HIBADHB,ALDH2.2,ALDH9A1A.1,ALDH1B1,ACADS,HADHB |
Protein Function
BCKDHB 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 BCKDHB itself. We selected most functions BCKDHB had, and list some proteins which have the same functions with BCKDHB. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| alpha-ketoacid dehydrogenase activity | BCKDHA |
| carboxy-lyase activity | AMD2,LOC650128,PDXDC1,PDXDC2P,ECHDC1,MVDA,SGPL1,GAD1B,BCKDHA,URAD |
| 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity | BCKDHA |
| protein binding | CASQ2,C14orf135,Car4,AIMP2,KIRREL,C16orf5,XRCC5,ENPP1,CEP164,PDLIM3 |
Interacting Protein
BCKDHB 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 BCKDHB here. Most of them are supplied by our site. Hope this information will be useful for your research of BCKDHB.
BCKDHA;HDAC6;Hdac6
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