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mitochondrial encephalomyopathy

" in MedChemExpress (MCE) Product Catalog:
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Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-175739
    TFAM activator 1

    		Mitochondrial Metabolism CXCR Neurological Disease Metabolic Disease Inflammation/Immunology
    TFAM activator 1 is a TFAM activator. TFAM activator 1 increases TFAM protein levels, promotes mitochondrial DNA stability, increases mitochondrial DNA copy number, and prevents mitochondrial DNA from escaping into the cytoplasm. TFAM activator 1 improves cellular energy metabolism in cybrid cells. TFAM activator 1 reduces fibrosis markers in fibroblasts. TFAM activator 1 can be used in the research of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome, systemic sclerosis, and autoimmune diseases .
    TFAM activator 1
  • HY-128895A
    KL1333 hydrochloride

    		Mitochondrial Metabolism Quinone Reductase Metabolic Disease
    KL1333 hydrochloride is an orally active NAD + modulator that reacts with NAD(P)H:quinone oxidoreductase 1 (NQO1) as a substrate, leading to increased intracellular NAD + levels through NADH oxidation. Elevated NAD + levels trigger activation of SIRT1 and AMPK, and subsequently activate PGC-1α. KL1333 hydrochloride improves energy metabolism and mitochondrial dysfunction in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) fibroblasts. KL1333 hydrochloride protects against cisplatin-induced ototoxicity in mouse cochlear cultures .
    KL1333 hydrochloride
  • HY-149193
    5-Taurinomethyluridine

    		Endogenous Metabolite Metabolic Disease
    5-Taurinomethyluridine is a taurine-containing modified uridine and translation regulator that exists in Trp and Leu (UUR) tRNAs of mammalian mitochondria. 5-Taurinomethyluridine is located at the first position of the anticodon of these mitochondrial tRNAs. 5-Taurinomethyluridine is synthesized from taurine and one-carbon metabolites by GTPBP3/MTO1, and its deficiency directly causes abnormal mitochondrial translation and various human mitochondrial diseases .
    5-Taurinomethyluridine

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