Human BCKDHA ELISA Kit
Sensitivity
2.2 pg/mL
Range
39-2500 pg/mL
Reactivity
Human
Cat no : KE01083
Synonyms
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, BCKDH E1-alpha, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, EC:1.2.4.4, MSU
Validation Data Gallery
Product Information
KE01083 is a solid phase sandwich Enzyme Linked-Immuno-Sorbent Assay (Sandwich ELISA). The human BCKDHA ELISA kit is to be used to detect and quantify protein levels of endogenous human BCKDHA. The assay recognizes human BCKDHA. An antibody specific for human BCKDHA has been pre-coated onto the microwells. The human BCKDHA protein in samples is captured by the coated antibody after incubation. Following extensive washing, another horseradish peroxidase (HRP)-conjugated antibody specific for human BCKDHA is added to detect the captured human BCKDHA protein. For signal development, followed by Tetramethyl-benzidine (TMB) reagent. Solution containing sulfuric acid is used to stop color development and the color intensity which is proportional to the quantity of bound protein is measurable at 450 nm with the correction wavelength set at 630 nm.
| Product name | Human BCKDHA ELISA Kit |
| Tests | 1 X 96 well plate |
| Sample type | Cell lysate |
| Assay type | Sandwich |
| Sensitivity | 2.2 pg/mL |
| Range | 39-2500 pg/mL |
| Reactivity | Human |
| Tested applications | Sandwich ELISA |
| Gene ID (NCBI) | 593 |
Recovery
| Sample Type | Average | Range |
|---|---|---|
| Cell lysate | 81% | 78%-85% |
IntraAssay
| Sample | n | mean ( pg/mL) | SD | CV% |
|---|---|---|---|---|
| 1 | 8 | 1,219.4 | 75.5 | 6.2 |
| 2 | 8 | 301.0 | 15.0 | 5.0 |
| 3 | 8 | 149.6 | 5.6 | 3.7 |
InterAssay
| Sample | n | mean ( pg/mL) | SD | CV% |
|---|---|---|---|---|
| 1 | 16 | 1,226.5 | 68.7 | 5.6 |
| 2 | 16 | 310.0 | 18.0 | 5.8 |
| 3 | 16 | 164.1 | 16.4 | 10.0 |
Background Information
BCKDHA (branched-chain ketoacid dehydrogenase E1 subunit alpha) is a mitochondrial enzyme subunit that forms part of the branched-chain α-ketoacid dehydrogenase (BCKDH) complex, which catalyzes the rate-limiting step in the catabolism of branched-chain amino acids (BCAAs), including leucine, isoleucine, and valine. BCKDHA encodes the E1α catalytic subunit, which functions as a heterotetramer with E1β and mediates the oxidative decarboxylation of branched-chain α-ketoacids. Its activity is tightly regulated by phosphorylation-dephosphorylation, linking BCKDHA to metabolic homeostasis. Dysregulation or mutation of BCKDHA leads to impaired BCAA metabolism and is a primary cause of maple syrup urine disease (MSUD). Emerging evidence also implicates altered BCKDHA activity in cancer metabolism, insulin resistance, and mitochondrial dysfunction, highlighting its role beyond inherited metabolic disorders.
Properties
| Storage Instructions | All the reagents are stored at 2-8℃ for 6 months or -20℃ for 12 months. Refer to the protocol for further storage instructions. |
| Synonyms | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, BCKDH E1-alpha, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, EC:1.2.4.4, MSU |