Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes.

•We describe an organotypic liver model for propionic acidemia using patient derived hepatocytes.•Hepatocytes in model retained differentiated morphology and function stably over 10days.•Molecular enzymatic defect was stably retained at gene and protein level.•Increased ammonia production by patient derived hepatocytes in response to amino acid loading in model•Similar responses not exhibited by healthy donor hepatocytes in model