Mechanism of Pyridoxine 5′-Phosphate Accumulation in Pyridoxal 5′-Phosphate-Binding Protein Deficiency
Tomokazu Ito, Honoka Ogawa, Hisashi Hemmi, Diana M. Downs, Tohru Yoshimura
Abstract
homeostasis and perturbs diverse metabolisms, including amino acid and α-keto acid metabolism. Accumulation of PNP is a characteristic phenotype of PLPBP deficiency and is suggested to be a potential cause of the pleiotropic effects, but the mechanism of this accumulation has been poorly understood. In this study, we show that fluxes for PNP synthesis/metabolism are not responsible for the accumulation of PNP. Our results indicate that PLPBP is involved in the homeostasis of pyridoxamine 5'-phosphate, and that its disruption may lead to the accumulation of PNP in PLPBP deficiency.
Topics & Concepts
PyridoxalPyridoxineBiochemistryBiologyIntracellularPyridoxine DeficiencyAmino acidPyridoxal phosphateEscherichia coliEnzymeHomeostasisVitaminMetabolic pathwayFlux (metallurgy)MetabolismBiosynthesisOxidase testSecretionPhenotypeProtein biosynthesisCell biologyBinding proteinB vitaminsEnzyme Structure and FunctionMetabolism and Genetic DisordersFolate and B Vitamins Research