Ask about this productRelated genes to: BCKDHA Blocking Peptide
- 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 Blocking Peptide
Related articles to: BCKDHA Blocking Peptide
- Adaptive thermogenesis in beige adipocytes is essential for maintaining energy homeostasis and preventing obesity. Emerging evidence suggests that human visceral adipose tissue harbors adipocytes with beige-like thermogenic features, enabling analysis of thermogenic gene regulation in humans. Isocitrate dehydrogenase 3A (IDH3A) is a rate-limiting enzyme of the tricarboxylic acid cycle, yet its function in adipocytes remains poorly defined. In this study, we examined IDH3A expression in human visceral adipose tissue and generated adipocyte-specific IDH3A knockout mice to investigate its role in beige adipocyte thermogenesis and metabolic regulation. IDH3A expression in human visceral fat was inversely associated with adiposity and adverse metabolic traits. Moreover, IDH3A expression was induced in human and mouse adipocytes following thermogenic stimulation. Adipocyte-specific IDH3A deletion in mice impaired beige fat thermogenic capacity, led to cold intolerance, and exacerbated diet-induced metabolic dysfunction. Mechanistically, IDH3A deficiency increased DNA methylation at the Bckdha promoter, resulting in the repression of this key branched-chain amino acid (BCAA) catabolic gene and impaired BCAA catabolism. Notably, restoring BCKDHA in IDH3A-deficient adipocytes rescued respiration and thermogenic function. Together, in addition to its canonical enzymatic role, our findings identify IDH3A as a critical regulator of BCAA catabolism that facilitates adaptive thermogenesis under metabolic stress conditions. - Source: PubMed
Lu XuhongBai NingningLyu JialuSun JingjingSu YingyingYe YafenHu TingtingLiu TianwenLi WenfeiLi XiaoyaMa XiaojingYang YingBao Yuqian - Metabolic dysfunction-associated steatotic liver disease (MASLD) is a common chronic liver disease. This study investigates the anti-MASLD effects of dietary prebiotic stachyose (STA) on disease progression identifying Butyricimonas virosa as a key bacterium boosted by STA supplementation. Oral gavage of B. virosa to high fat diet (HFD)-fed mice significantly suppresses the progression of MASLD and modulates gut microbiota composition. Integration of metagenomic and metabolomic data demonstrates that B. virosa treatment significantly enhances the production of thiamine monophosphate (TMP), as well as its conversion to thiamine and subsequent accumulation in the liver. The accumulation of hepatic thiamine further leads to elevated thiamine pyrophosphate (TPP) concentrations enhancing the activity of branched-chain α-keto acid dehydrogenase E1 subunit α (BCKDHA) associated with augmented degradation of branched chain amino acids (BCAAs). Administration of B. virosa compensates via production of gut bacterial-derived TMP for hepatic TPP deficiency in mice fed a thiamine-deficient HFD. A population-based analysis reveals an inverse correlation between plasma thiamine levels, abundances of bacterial genes involved in thiamine synthesis and metabolism, and phenotypes associated with MASLD, suggesting that key genes involved in fecal thiamine metabolism, as well as serum thiamine determination, may potentially serve as biomarkers for the diagnosis of MASLD. - Source: PubMed
Publication date: 2026/01/20
He NingningWang HaoyuYang ZizhenLi HuiLiu BeiChen KaiweiWu ZhinanZhao XinnanLiang HeweiWang MengmengLi XiaofangZhong YiyiZhang HaifengXiao LiangKristiansen KarstenPeng JixingZou YuanqiangLi Shangyong - Recent research has emphasized the significance of testis-specific serine proteases in regulating various aspects of sperm maturation and function. Among them, serine protease 55 (PRS55) plays an important role in the energy metabolism of sperm and is essential for male fertility in mice. A recent case study further suggests its potential importance to human fertility. However, the underlying molecular mechanism by which PRS55 influences sperm function are still not well understood. The present study aims to investigate these mechanisms further. - Source: PubMed
Publication date: 2025/12/24
Ge HaoyangShen ChunlingXiong WenfengLu ShunyuanLi ChaojieTang LingyunShen YanZhang HongxinFei JianWang Zhugang - Maple syrup urine disease (MSUD) is a rare inherited metabolic disorder caused by a deficiency in the branched-chain alpha-keto acid dehydrogenase complex, leading to the accumulation of branched-chain amino acids. This case series describes the clinical and genetic findings of ten Iranian patients with MSUD, focusing on novel mutations in the BCKDHA, BCKDHB, and DBT genes. - Source: PubMed
Publication date: 2025/12/11
Jafari MansoorehKarami FatemehSalahshourifar ImanSetoodeh AriaRahmanifar AliBagherian HamidehAlaei Mohammad RezaRohani FarzanehZeinali Sirous - Reprogrammed cellular metabolism plays a critical role in the development and progression of various cancers. However, the mechanisms by which these metabolic changes drive malignancy in gallbladder cancer (GBC) remain unclear. In this study, we identified significant alterations in branched-chain amino acid (BCAA) metabolism in GBC through comprehensive transcriptomic and metabolic analyses. Reduced activity of the BCAA catabolic enzymes ACADS, ACADSB, and BCKDHA was associated with poor prognosis in GBC patients. Dietary reduction of BCAAs in mouse model of GBC significantly slowed tumor growth. The lncRNA LPAL2 was found to correlate with the expression of BCAA catabolic enzymes. In vivo and in vitro assays demonstrated that LPAL2 inhibited GBC cell proliferation, downregulated intracellular BCAA levels, and suppressed mTORC1 activation. Furthermore, LPAL2 and ACADS were identified as independent prognostic factors for survival. Mechanically, IGF2BP1 maintaining LPAL2 stability through methylation. Additionally, LPAL2 decreased YBX1 stability by promoting its ubiquitination-mediated degradation, while YBX1 inhibited the transcription of certain BCAA catabolic enzymes through binding to their promoters. In summary, the LPAL2-YBX1 interaction regulates BCAA metabolism to influence GBC cell proliferation, which could be targeted for therapeutic interventions in GBC treatment. - Source: PubMed
Publication date: 2025/12/08
Li XuechuanLiu KeLi LinYan SiyuanYang YueYang YangYang JiahuaZou LuLi WeijianLi GuoqiangYang MaoLiu LiguoWang ZiyiGeng YajunGong WeiLiu YingbinWu Xiangsong