product summary
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company name :
MyBioSource
product type :
ELISA/assay
product name :
Mouse 2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial (BCKDHA) ELISA Kit
catalog :
MBS9360708
quantity :
48-Strip-Wells
price :
470 USD
more info or order :
product information
catalog number :
MBS9360708
products type :
ELISA Kit
products full name :
Mouse 2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial (BCKDHA) ELISA Kit
products short name :
[2-Oxoisovalerate Dehydrogenase Subunit Alpha, Mitochondrial (BCKDHA)]
other names :
[2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 2; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 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
reactivity :
Mouse
specificity :
No significant cross-reactivity or interference between this analyte and analogues is observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Assay Type: Quantitative Sandwich. Detection Range: 0.625 ng/ml - 20 ng/ml. Sensitivity: 0.1 ng/ml.
other info2 :
Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
products description :
Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of BCKDHA (hereafter termed "analyte") in undiluted original Mouse body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.
ncbi gi num :
258645172
ncbi acc num :
NP_001158255.1
ncbi gb acc num :
NM_001164783.1
ncbi mol weight :
50,790 Da
ncbi pathways :
2-oxobutanoate Degradation 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 (1270161); Glyoxylate Metabolism And Glycine Degradation Pathway (1270180); L-isoleucine Degradation I Pathway (139163); L-leucine Degradation I Pathway (139165); L-threonine Degradation V Pathway (1108792); L-valine Degradation I Pathway (139219)
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: Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Oxidoreductase; EC 1.2.4.4. Chromosomal Location of Human Ortholog: 19q13.1-q13.2. Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion. Molecular Function: 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; alpha-ketoacid dehydrogenase activity; carboxy-lyase activity; metal ion binding; protein binding. Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process. Disease: Maple Syrup Urine Disease
size1 :
48-Strip-Wells
price1 :
470 USD
size2 :
96-Strip-Wells
price2 :
680
size3 :
5x96-Strip-Wells
price3 :
3100
size4 :
10x96-Strip-Wells
price4 :
6095
more info or order :
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.
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