BCKDHA predesign siRNA
- Known as:
- BCKDHA predesign small interfearing RNA
- Catalog number:
- RI10559
- Category:
- -
- Supplier:
- Abgen
- Gene target:
- BCKDHA predesign siRNA
Related genes to: BCKDHA predesign siRNA
- 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 predesign siRNA
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- 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 - 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