Ureidopropionic acid

Ureidopropionic acid (3-Ureidopropionic acid) is a selective mitochondrial respiratory chain complex V inhibitor. 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^[1]. In Vitro:Ureidopropionic acid (0.001-10 mM; 1-24 h) induces concentration- and time-dependent neurodegeneration in primary chick embryo telencephalon neurons, enhances L-glutamate-mediated excitotoxicity, and this neurotoxicity is partially mitigated by α-tocopherol or MK-801 (HY-15084B) pretreatment^[1].
Ureidopropionic acid (1 mM; 0.5-24 h) induces a delayed, time-dependent increase in intracellular calcium concentrations in primary chick embryo telencephalon neurons, which is partially attenuated by MK-801^[1].
Ureidopropionic acid (0.01-1 mM; 0.5-24 h) induces concentration- and time-dependent increases in mitochondrial ROS production in primary chick embryo telencephalon neurons, and this ROS generation is not inhibited by MK-801^[1].
Ureidopropionic acid specifically inhibits the activity of mitochondrial respiratory chain complex V (but not complexes I-IV) in bovine heart submitochondrial particles, with concentration-dependent inhibition observed^[1].