Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA) | MyBioSource MBS952592 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

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

catalog :

MBS952592

quantity :

0.5 mg (E-Coli)

price :

1170 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS952592

products type :

Recombinant Protein

products full name :

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

products short name :

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA)

products name syn :

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; EC=1.2.4.4; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha

other names :

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDH E1-alpha; 2-oxoisovalerate dehydrogenase (lipoamide); branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; branched chain keto acid dehydrogenase E1, alpha polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha

products gene name :

BCKDHA

other gene names :

BCKDHA; BCKDHA; MSU; MSUD1; OVD1A; BCKDE1A; BCKDE1A; BCKDH E1-alpha

uniprot entry name :

ODBA_HUMAN

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

46-445

sequence length :

400

sequence :

SSLDD KPQFPGASAE FIDKLEFIQP NVISGIPIYR VMDRQGQIIN PSEDPHLPKE KVLKLYKSMT LLNTMDRILY ESQRQGRISF YMTNYGEEGT HVGSAAALDN TDLVFGQYRE AGVLMYRDYP LELFMAQCYG NISDLGKGRQ MPVHYGCKER HFVTISSPLA TQIPQAVGAA YAAKRANANR VVICYFGEGA ASEGDAHAGF NFAATLECPI IFFCRNNGYA ISTPTSEQYR GDGIAARGPG YGIMSIRVDG NDVFAVYNAT KEARRRAVAE NQPFLIEAMT YRIGHHSTSD DSSAYRSVDE VNYWDKQDHP ISRLRHYLLS QGWWDEEQEK AWRKQSRRKV MEAFEQAERK PKPNPNLLFS DVYQEMPAQL RKQQESLARH LQTYGEHYPL DHFDK

purity :

>90%

form :

Liquid containing glycerol; lyophilization may be available upon request.

storage stability :

Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

other info1 :

Species: Homo sapiens (Human)

products description :

BCKDHA

ncbi gi num :

11386135

ncbi acc num :

NP_000700.1

ncbi gb acc num :

NM_000709.3

uniprot acc num :

P12694

ncbi mol weight :

50,471 Da

ncbi pathways :

2-oxobutanoate Degradation I Pathway (142413); 2-oxobutanoate Degradation I Pathway (139464); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (142335); 2-oxoisovalerate Decarboxylation To Isobutanoyl-CoA Pathway (139118); Branched-chain Amino Acid Catabolism Pathway (106179); Leucine Degradation, Leucine = Acetoacetate + Acetyl-CoA Pathway (413354); Leucine Degradation, Leucine = Acetoacetate + Acetyl-CoA Pathway (468229); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Valine, Leucine And Isoleucine Degradation Pathway (82952)

ncbi summary :

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.[provided by RefSeq, Sep 2009]

uniprot summary :

BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation. Protein type: Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.2.4.4; Lyase; Mitochondrial. Chromosomal Location of Human Ortholog: 19q13.1-q13.2. Cellular Component: mitochondrion; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix. Molecular Function: protein binding; alpha-ketoacid dehydrogenase activity; metal ion binding; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity. Biological Process: branched chain family amino acid catabolic process. Disease: Maple Syrup Urine Disease

size1 :

0.5 mg (E-Coli)

price1 :

1170 USD

size2 :

0.05 mg (Baculovirus)

price2 :

1170

size3 :

0.5 mg (Yeast)

price3 :

1390

size4 :

0.05 mg (Mammalian-Cell)

price4 :

1390

size5 :

1 mg (E-Coli)

price5 :

1880

more info or order :

MyBioSource product webpage