BCKDHA antibody
- Known as:
- BCKDHA (anti-)
- Catalog number:
- orb37075
- Product Quantity:
- EUR
- Category:
- -
- Supplier:
- Biorbyt biorb
- Gene target:
- BCKDHA antibody
Related genes to: BCKDHA antibody
- Gene:
- BCKDHA NIH gene
- Name:
- branched chain keto acid dehydrogenase E1, alpha polypeptide
- Previous symbol:
- OVD1A
- Synonyms:
- MSU
- Chromosome:
- 19q13.2
- Locus Type:
- gene with protein product
- Date approved:
- 1989-06-30
- Date modifiied:
- 2016-10-05
Related products to: BCKDHA antibody
Related articles to: BCKDHA antibody
- Head and neck squamous cell carcinoma (HNSCC) poses a significant global health challenge, characterized by low survival rates and frequent metastasis. Conventional therapies are often restricted by systemic toxicity and drug resistance, positioning natural bioactive extracts like Total Glucosides of Paeony (TGP) as promising therapeutic alternatives. While the amino acid transporter SLC7A5 is known to drive tumor proliferation by facilitating leucine uptake and activating the mTOR signaling pathway, the specific interaction between TGP and this SLC7A5/mTOR axis in HNSCC has not been fully elucidated. - Source: PubMed
Publication date: 2026/07/11
Li YuanqiangHu GaofengNiu WenyuanZhang LizhuoGe JiamingXuan JieYu XuefeiMa ShangLi MengjiaZhang ZiqiangLuo KeJia XingLi Qinglin - Although the hearts of large animals are known to exhibit regional heterogeneity in structure, function, and metabolism, their spatial patterns of gene expression remain poorly understood. Therefore, we investigated regional differences in gene expression across six anatomical regions of the bovine heart, including the atria, ventricles, interventricular septum, and apex ( = 3). The expression levels of genes related to cardiac development, structure, and branched-chain amino acid (BCAA) metabolism were analyzed using quantitative real-time PCR, and multivariate patterns were evaluated using principal component analysis (PCA). Histological analysis suggested no apparent structural differences among the examined regions. expression showed no significant regional variation, suggesting conserved cardiomyocyte identity. Conversely, contractile genes (, , , and ) and the BCAA metabolism-related gene were significantly more highly expressed in the ventricular myocardium than in the atrial regions. PCA clearly distinguished between the atrial and ventricular myocardium and indicated coordinated variations in contractile and metabolic gene expression. These findings of region-specific molecular heterogeneity in the bovine heart, characterized by regional differences in gene expression across the myocardium rather than morphological differences, provide new insights into the spatial regulation of cardiac function and highlight the value of the bovine heart as a model for studying myocardial specialization. - Source: PubMed
Publication date: 2026/07/01
Takiguchi RinWatanabe KenichiDoai YutsukiHashimoto MisuzuWatanabe HiroyukiMuranishi Yuki - Neoadjuvant chemoradiotherapy (nCRT) resistance in locally advanced rectal cancer (LARC) leads to worse outcomes. The present pilot study aimed to identify candidate proteins associated with resistance to nCRT. Data-independent acquisition mass spectrometry on 15 formalin-fixed paraffin-embedded tumor tissues, collected before and after nCRT, from four nCRT-resistant and four nCRT-sensitive LARC cases, was performed, identifying 6,006 proteins. Before nCRT, 133 proteins were found to be more abundant in resistant tumors and were preliminarily enriched in cell junction and metabolic pathways. After nCRT, 290 proteins were found to be more abundantly expressed in resistant tumors and were preliminarily enriched in pathways associated with DNA replication, transcription, translation and metabolism. Combined analysis of datasets GSE209746 and PXD060201 prioritized branched-chain keto acid dehydrogenase E1 subunit α (BCKDHA) as a candidate resistance-associated protein. In a colorectal cancer (CRC) tissue microarray, upregulated BCKDHA expression was associated with shorter overall survival, supporting its broader clinical relevance in CRC. In SW480 cells, BCKDHA overexpression increased clonogenic survival following irradiation, whereas BCKDHA knockdown enhanced radiosensitivity. These changes were accompanied by altered ataxia telangiectasia mutated, checkpoint kinase 2, DNA repair protein RAD51 homolog 1-associated signaling and γ-H2A histone family member X dynamics after irradiation. Collectively, these data identified BCKDHA as a candidate nCRT resistance-associated protein in LARC and provided preliminary functional evidence associating BCKDHA with the response to radiotherapy and DNA damage response-associated signaling. Further validation in larger pretreatment LARC cohorts and more direct mechanistic studies is warranted. - Source: PubMed
Publication date: 2026/06/24
He Jiang-YiDong YanChen CanLu Fang-HaoRan Feng-WeiDing QiongDu YunLiu Tao-RuiZhang Yu-YingWang TengChen Yu-HanZhao XiangXu Sen-LinLi Jian-Jun - Excessive fructose intake is a major dietary factor that promotes hepatic steatosis. Branched-chain amino acid (BCAA) catabolism has recently been implicated in hepatic lipid homeostasis, but whether fructose overload affects hepatocyte BCAA catabolism and whether this contributes to fructose-induced lipid dysregulation remain unclear. Here, we examined hepatic BCAA metabolic responses to fructose using acute fructose gavage, intraperitoneal fructose administration, primary hepatocyte stimulation, and hepatocyte-specific Bckdha-deficient mice. Acute fructose gavage rapidly altered hepatic BCAA catabolic markers, and this effect was recapitulated by intraperitoneal fructose administration, indicating that systemic fructose exposure is sufficient to perturb hepatic BCAA metabolism. Consistently, fructose suppressed BCAA catabolic gene expression in isolated primary hepatocytes. Functionally, hepatocyte-specific Bckdha deficiency aggravated fructose-induced hepatic lipid accumulation and enhanced the expression of genes involved in de novo lipogenesis, including Srebf1, Acaca, Fasn, Scd1, and Elovl6. Collectively, these findings suggest that fructose overload suppresses hepatocyte BCAA catabolism and that impaired BCAA catabolism amplifies fructose-induced hepatic lipogenic programs and lipid accumulation. - Source: PubMed
Publication date: 2026/05/20
Zhong MengyuLuo XinyuShi MengliZhang Weiping JNiu WenyanSun HaipengZhang Xuejiao - Branched-chain 2-ketoacid dehydrogenase (BCKDH) deficiency (maple syrup urine disease; MSUD) causes lethal encephalopathy by disrupting cerebral metabolism, a process imperfectly reflected by circulating biomarkers. Diet and liver transplantation stabilize peripheral metabolites but fail to restore brain neurochemistry, demarcating the central nervous system as the decisive therapeutic compartment. To define the pathogenesis of intoxication and its therapeutic response, we performed paired serum-brain metabolomics in Bckdha-/- mice treated with a systemic AAV9 dual-gene vector encoding human BCKDHA and BCKDHB (A-BiP-B). Untreated neonates exhibited a 9-fold elevation of brain 2-ketoisocaproate accompanied by cerebral depletion of glutamate and glutamine, as well as shifts in tricarboxylic acid cycle and ketone body metabolism. These disturbances originated from reversal of branched-chain aminotransferase 2 flux and destabilization of glutamate-2-ketoglutarate mass balance, producing divergent metabolic endophenotypes in blood versus brain. A single intravenous injection of A-BiP-B rescued mice from fatal encephalopathy, partially restored cerebral BCKDHA mRNA expression, and brought core brain neurochemical endpoints within wild-type range despite persistent elevation of serum 2-ketoacids. These findings expose limitations of current MSUD management and establish systemic dual-gene therapy as a means of restoring neurochemical homeostasis while enabling survival on unrestricted protein intake. - Source: PubMed
Publication date: 2026/05/13
Wang JiamingTurgeon Coleman TLoken Perry RGray-Edwards HeatherGao GuangpingTortorelli SilviaWang DanStrauss Kevin A