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Official Full Name branched chain keto acid dehydrogenase E1, beta polypeptide
Background 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
    • Species :
    • Chicken
    • Human
    • Mouse
    • Rat
    • Zebrafish
    • Source :
    • E.coli
    • HEK293
    • Mammalian Cell
    • Wheat Germ
    • Tag :
    • GST
    • His
    • N/A
    Species Cat.# Product name Source (Host) Tag Protein Length Price
    Human BCKDHB-10173H Recombinant Human BCKDHB, GST-tagged E.coli GST
    Human BCKDHB-137H Recombinant Human BCKDHB Protein, GST-tagged Wheat Germ GST
    Human BCKDHB-8492HCL Recombinant Human BCKDHB 293 Cell Lysate HEK293 N/A
    Human BCKDHB-8493HCL Recombinant Human BCKDHB 293 Cell Lysate HEK293 N/A
    Mouse BCKDHB-2332M Recombinant Mouse BCKDHB Protein Mammalian Cell His
    Rat BCKDHB-954R Recombinant Rat BCKDHB Protein Mammalian Cell His
    Chicken BCKDHB-6225C Recombinant Chicken BCKDHB Mammalian Cell His
    Zebrafish BCKDHB-5133Z Recombinant Zebrafish BCKDHB Mammalian Cell His

    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
    Valine, leucine and isoleucine degradation AACS; HIBCH; ALDH9A1B; PCCA; ALDH7A1; ACAT1; ACAT2; OXCT1; AOX2P; AUH
    Propanoate metabolism SUCLA2; DLDH; LDHA; HIBCH; ALDH6A1; ACACA; PCCA; BCKDHA; LDHC; MUT
    Metabolic pathways ACAA2; DPAGT1; CKBB; ATP5B; PDHA1B; ACADVL; PIP5K1BA; INPP4B; ALDH2.2; UOX

    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
    3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity BCKDHB; BCKDHA
    alpha-ketoacid dehydrogenase activity BCKDHB; BCKDHA
    carboxy-lyase activity LOC650128; PDXDC1; BCKDHA; MVDA; PDXDC2P; GAD1B; URAD; AMD2; BCKDHB; SGPL1
    protein binding SLC39A13; EPHA3; ZC3H18; NAT10; DENR; PIAS2; TMEM106B; MOCS3; MALSU1; C14orf166

    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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