Patient iPSC-derived astrocytes reveal impaired homeostasis and reactive-like features in propionic acidemia.
Propionic acidemia (PA) is a neurometabolic disorder caused by propionyl-CoA carboxylase deficiency with frequent neurological involvement, yet cell-type-specific mechanisms remain poorly defined. We established human induced pluripotent stem cell (iPSC)-derived astrocytes (iAs) from patients harboring PCCA or PCCB mut...