PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
- Product namePDH E1 alpha protein (PDHA1) ELISA KitSee all Pyruvate Dehydrogenase E1-alpha subunit kits ...
Intra-assay Sample n Mean SD CV% 1 8 = 2.3% Inter-assay Sample n Mean SD CV% 1 4 = 8.2%
- Tests1 x 96 test
- Sample typeCell culture extracts, Tissue Extracts
- Assay typeQuantitative
- Sensitivity= 15 ng/ml
- Species reactivityReacts with: Mouse, Rat, Cow, Human
- Product overview
ab115342 is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of PDHA1 protein in cell and tissue lysates. The assay employs an antibody specific for PDHA1 protein coated on a 96-well plate. Standards and samples are pipetted into the wells and PDHA1 protein present in the sample is bound to the wells by the immobilized antibody. The wells are washed and a biotin labeled anti-PDHA1 protein detector antibody is added. After washing away unbound detector antibody, HRP-conjugated streptavidin specific for the biotin labeled detector antibody is pipetted into the wells. The wells are again washed, an HRP substrate solution (TMB) is added to the wells and color develops in proportion to the amount of PDHA1 protein bound. The developing blue color is measured at 600 nm. Optionally the reaction can be stopped by adding hydrochloric acid which changes the color from blue to yellow and the intensity can be measured at 450 nm.
Store all components at 4°C. This kit is stable for at least 6 months
from receipt. After reconstitution the standard should be stored at -
80°C. Unused microplate strips should be returned to the pouch
containing the desiccant and resealed.
- Tested applicationsELISA more details
- Storage instructionsStore at +4°C. Please refer to protocols.
Components 1 x 96 tests 10X Blocking Buffer 1 x 6ml 10X HRP Label 1 x 1ml 10X PDHA1 protein Detector Antibody 1 x 1ml 20X Buffer 1 x 20ml Extraction Buffer 1 x 15ml Human PDHA1 protein standard (1 ug) 1 vial Microplate 96 antibody coated wells in 12 strips 1 unit TMB Development Solution 1 x 12ml
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Energy Metabolism
Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.
modificationsPhosphorylation at Ser-293 by PDK family kinases blocks the access to active site, and inactivates the enzyme.
- PDHA1PDHE1-A type IPyruvate dehydrogenase E1 component subunit alphasomatic form
Our Abpromise guarantee covers the use of ab115342 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
PDH E1 alpha protein (PDHA1) ELISA Kit images
Example of standard curve constructed using ab115342.
Example HeLa dilution series and working range produced with ab115342.
An experiment to determine the levels of PDHA1 in various fibroblast cell lines. A fibroblast cell line (P) harboring a mutation in the X-chromosome PDHA1 gene results in significant reduction in E1a protein expression. This is compared to control adult fibroblast cells (control) and mouse fibroblast cells (NIH3T3 and MEF, mouse embryonic fibroblasts).
References for PDH E1 alpha protein (PDHA1) ELISA Kit (ab115342)
ab115342 has not yet been referenced specifically in any publications.