This strain is a propionic acidemia (PA) disease model generated in the FVB/NJCya background strain using gene editing technology to replace a portion of the mouse Pcca gene sequence with the coding sequence (CDS) of the human PCCA gene carrying the A138T mutation. This model, by simultaneously knocking out the endogenous mouse Pcca gene and expressing the mutant human PCCA gene, mimics the metabolic characteristics of human PA patients and is suitable for investigating the pathogenic mechanisms and therapeutic interventions for propionic acidemia.