Mitochondria are recognized as one of the most important targets for new drug design in
cancer, cardiovascular, and neurological diseases. Currently, the most effective way to …

Propionyl-CoA carboxylase (PCC) is the enzyme which catalyzes the carboxylation of
propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form …

Propionic acidemia/aciduria (PA) is an ultra-rare, life-threatening, inherited metabolic
disorder caused by deficiency of the mitochondrial enzyme, propionyl-CoA carboxylase …

Propionic acidemia (PA) is a genetic metabolic disorder caused by mutations in the
mitochondrial enzyme, propionyl-CoA carboxylase (PCC), which is responsible for …

Organic acidurias (OADs) are inherited neurometabolic diseases largely caused by
deficiencies in enzymes involved in amino acid degradation, which result in accumulation of …

Life-threatening hyperammonemia occurs in both inherited and acquired liver diseases
affecting ureagenesis, the main pathway for detoxification of neurotoxic ammonia in …

Boronate-based molecular probes are emerging as one of the most effective tools for
detection and quantitation of peroxynitrite and hydroperoxides. This review discusses the …

Propionic acidemia (PA) is a rare autosomal-recessive metabolic disease that arises from
mutations in propionyl-CoA (C3-CoA) carboxylase. Reduced enzyme activity slows C3-CoA …

Organic acidurias (OADs) comprise a biochemically defined group of inherited metabolic
diseases. Increasing awareness, reliable diagnostic work‐up, newborn screening programs …

Abstract Propionic acidemia (PA, OMIM 606054) is a devastating inborn error of metabolism
arising from mutations that reduce the activity of the mitochondrial enzyme propionyl‐CoA …