Human BCKDHA ELISA Kit (V2LY-0626-LY4154)

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Tested Data
Datasheet
SDS
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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Sensitivity
0.021 ng/mL
Detection Range
0.05-20 ng/mL
Sample Type
Serum, Plasma, cell culture supernates
Specificity
Human
Assay Type
Sandwich
Reactivity
Human
Assay Time
1.5 h
Molecule Mass
50.5 kDa
Components
  • Pre-coated ELISA plate: 12 wells * 8 detachable strips
  • Standard solution: 0.5ml x1
  • Standard diluent: 3ml x1
  • Streptavidin-HRP: 6ml x1
  • Stop solution: 6ml x1
  • Substrate solution A: 6ml x1
  • Substrate solution B: 6ml x1
  • Wash buffer concentrate (25x): 20ml x1
  • Biotinylated antibody: 1ml x1

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 2-8°C
More Infomation

Target

Full Name
branched chain keto acid dehydrogenase E1, alpha polypeptide
Function
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Biological Process
Branched-chain amino acid catabolic process Source: HGNC-UCL
Cellular Location
Mitochondrion matrix
Involvement in disease
Maple syrup urine disease 1A (MSUD1A): A metabolic disorder due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration. Clinical features include mental and physical retardation, feeding problems, and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine. If untreated, maple syrup urine disease can lead to seizures, coma, and death. The disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still involve developmental delay and other medical problems if not treated.

Knapik-Czajka, M., Jurczyk, M., Bieleń, J., Aleksandrovych, V., Gawędzka, A., Stach, P., ... & Gil, K. (2021). Effect of 5-fluorouracil on branched-chain α-keto acid dehydrogenase (BCKDH) complex in rat’s heart. Folia Medica Cracoviensia, 121-129.

Biswas, D., Dao, K. T., Mercer, A., Cowie, A., Duffley, L., El Hiani, Y., ... & Pulinilkunnil, T. (2020). Silencing branched-chain ketoacid dehydrogenase or treatment with branched-chain ketoacids ex vivo inhibits muscle insulin signaling. bioRxiv.

Biswas, D., Dao, K. T., Mercer, A., Cowie, A. M., Duffley, L., El Hiani, Y., ... & Pulinilkunnil, T. (2020). Branched-chain ketoacid overload inhibits insulin action in the muscle. Journal of Biological Chemistry, 295(46), 15597-15621.

Webb, L. A., Sadri, H., Von Soosten, D., Dänicke, S., Egert, S., Stehle, P., & Sauerwein, H. (2019). Changes in tissue abundance and activity of enzymes related to branched-chain amino acid catabolism in dairy cows during early lactation. Journal of dairy science, 102(4), 3556-3568.

Ali, E. Z., & Ngu, L. H. (2018). Fourteen new mutations of BCKDHA, BCKDHB and DBT genes associated with maple syrup urine disease (MSUD) in Malaysian population. Molecular genetics and metabolism reports, 17, 22-30.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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