Catalog No.
YHC94501
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Leu97-Lys400
Predicted molecular weight
36.75 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
P12694
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution. A copy of datasheet will be provided with the products, please refer to it for details.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial, 1.2.4.4, Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDE1A, BCKDH E1-alpha, BCKDHA
Integrated Amino Acid Profiling and 4D-DIA Proteomics Reveal Protein Quality Divergence and Metabolic Adaptation in Cordyceps Species., PMID:40422699
Advancing the Biochemical Understanding of Maple Syrup Urine Disease and the Impact of Liver Transplantation: A Pilot Study., PMID:40232068
Indispensable role of PGC1α signaling in lipid and carbohydrate metabolism of fish PPARα activation., PMID:40049462
Expanding the Genetic Spectrum of PPM1K-Related Maple Syrup Urine Disease: A Novel Mutation., PMID:40047138
BCKDHA-BCKDHB digenic gene therapy restores metabolic homeostasis in two mouse models and a calf with classic maple syrup urine disease., PMID:40009698
Glucose-dependent insulinotropic polypeptide/glucagon-like peptide 1 receptor agonist tirzepatide promotes branched chain amino acid catabolism to prevent myocardial infarction in non-diabetic mice., PMID:39928435
The pyruvate dehydrogenase kinase inhibitor dichloroacetate mitigates alcohol-induced hepatic inflammation and metabolic disturbances in mice., PMID:39621302
BCKDH kinase promotes hepatic gluconeogenesis independent of BCKDHA., PMID:39389936
A comprehensive in silico analysis of mutation spectrum of maple syrup urine disease (MSUD) genes in Iranian population., PMID:39315288
Adipocytes Are the Only Site of Glutamine Synthetase Expression Within the Lactating Mouse Mammary Gland., PMID:38813479
Transcriptomics and UHPLC-QQQ-MS analyses reveal the dysregulation of branched chain amino acids metabolism in renal fibrotic rats., PMID:38723558
Strategies to improve CHO cell culture performance: Targeted deletion of amino acid catabolism and apoptosis genes paired with growth inhibitor supplementation., PMID:38629737
Developing a novel immune infiltration-associated mitophagy prediction model for amyotrophic lateral sclerosis using bioinformatics strategies., PMID:38601155
Taohe Chengqi decoction alleviated metabolic-associated fatty liver disease by boosting branched chain amino acids catabolism in the skeletal muscles of type 2 diabetes mellitus., PMID:38387274
Reduced Branched-Chain Amino Acid Intake Improved High-Fat Diet-Induced Nonalcoholic Fatty Pancreas Disease in Mice., PMID:38227616
Branched-chain amino acid catabolic defect promotes α-cell proliferation via activating mTOR signaling., PMID:38158148
Branched-chain amino acid catabolic defect in vascular smooth muscle cells drives thoracic aortic dissection via mTOR hyperactivation., PMID:37956909
Enhancement of PPARα-Inhibited Leucine Metabolism-Stimulated β-Casein Synthesis and Fatty Acid Synthesis in Primary Bovine Mammary Epithelial Cells., PMID:37853551
PPM1K mediates metabolic disorder of branched-chain amino acid and regulates cerebral ischemia-reperfusion injury by activating ferroptosis in neurons., PMID:37752100
Branched-chain amino acid supplementation does not enhance lean tissue accretion in low birth weight neonatal pigs, despite lower Sestrin2 expression in skeletal muscle., PMID:37743429
Potential Association of Cytochrome P450 Copy Number Alteration in Tumour with Chemotherapy Resistance in Lung Adenocarcinoma Patients., PMID:37686184
BCKDHA contributes to melanoma progression by promoting the expressions of lipogenic enzymes FASN and ACLY., PMID:37377173
Identification of gene mutations in six Chinese patients with maple syrup urine disease., PMID:36911408
Successful treatment of severe MSUD in Bckdhb-/- mice with neonatal AAV gene therapy., PMID:36880392
Hepatic and serum branched-chain fatty acid profile in patients with nonalcoholic fatty liver disease: A case-control study., PMID:36876627
Dietary Leucine Supplementation Improves Muscle Fiber Growth and Development by Activating AMPK/Sirt1 Pathway in Blunt Snout Bream (Megalobrama amblycephala)., PMID:36860449
The mitochondrial BCKD complex interacts with hepatic apolipoprotein E in cultured cells in vitro and mouse livers in vivo., PMID:36749362
PPM1K defects cause mild maple syrup urine disease: The second case in the literature., PMID:36706222
Comprehensive analyses of the microRNA-messenger RNA-transcription factor regulatory network in mouse and human renal fibrosis., PMID:36457754
Branched-chain amino acid catabolism breaks glutamine addiction to sustain hepatocellular carcinoma progression., PMID:36417878
Genome-wide association and multi-trait analyses characterize the common genetic architecture of heart failure., PMID:36376295
Genomic and biochemical analysis of repeatedly observed variants in DBT in individuals with maple syrup urine disease of Central American ancestry., PMID:35799415
Neonatal gene therapy achieves sustained disease rescue of maple syrup urine disease in mice., PMID:35672312
Targeting BCAT1 Combined with α-Ketoglutarate Triggers Metabolic Synthetic Lethality in Glioblastoma., PMID:35499760
Three novel mutations of the BCKDHA, BCKDHB and DBT genes in Chinese children with maple syrup urine disease., PMID:34883003
BIX01294 inhibits EGFR signaling in EGFR-mutant lung adenocarcinoma cells through a BCKDHA-mediated reduction in the EGFR level., PMID:34876693
AICAR stimulates mitochondrial biogenesis and BCAA catabolic enzyme expression in C2C12 myotubes., PMID:34798200
Genetic analysis by targeted next-generation sequencing and novel variation identification of maple syrup urine disease in Chinese Han population., PMID:34556729
Expression of specific signaling components related to muscle protein turnover and of branched-chain amino acid catabolic enzymes in muscle and adipose tissue of preterm and term calves., PMID:34334194
Maple syrup urine disease: Characteristics of diagnosis and treatment in 45 patients in Chile., PMID:34288399
Neonatal maple syrup urine disease in China: two novel mutations in the BCKDHB gene and literature review., PMID:34187135
Pyridostigmine Protects Against Diabetic Cardiomyopathy by Regulating Vagal Activity, Gut Microbiota, and Branched-Chain Amino Acid Catabolism in Diabetic Mice., PMID:34084135
Challenges in Diagnosing Intermediate Maple Syrup Urine Disease by Newborn Screening and Functional Validation of Genomic Results Imperative for Reproductive Family Planning., PMID:34069211
Molecular basis of various forms of maple syrup urine disease in Chilean patients., PMID:33955723
Dysregulated Provision of Oxidisable Substrates to the Mitochondria in ME/CFS Lymphoblasts., PMID:33669532
Maple syrup urine disease: Clinical outcomes, metabolic control, and genotypes in a screened population after four decades of newborn bloodspot screening in the Republic of Ireland., PMID:33300147
Branched-chain ketoacid overload inhibits insulin action in the muscle., PMID:32878988
Effect of valine on myotube insulin sensitivity and metabolism with and without insulin resistance., PMID:32222880
Identification of eight novel mutations in 11 Chinese patients with maple syrup urine disease., PMID:32193832
For research use only. Not for human or drug use.
