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severe propionic aciduria

" in MedChemExpress (MCE) Product Catalog:
Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-113285

    3-Ureidopropionic acid

    Oxidative Phosphorylation Metabolic Disease
    Ureidopropionic acid is a mitochondrial respiratory chain complex V inhibitor that selectively inhibits the activity of mitochondrial respiratory chain complex V, with no effect on respiratory chain complexes I-IV or mitochondrial fatty acid β-oxidation. Ureidopropionic acid induces the production of reactive oxygen species, delayed elevation of intracellular calcium concentration, secondary energy-dependent excitotoxicity and neurodegeneration in neurons. Ureidopropionic acid promotes neuropathological changes by impairing mitochondrial energy metabolism, oxidative stress and excitotoxicity pathways. Ureidopropionic acid can be used in studies related to 3-ureidopropionase deficiency and severe propionic aciduria .
    Ureidopropionic acid

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