Figure 4
From: Acitretin mitigates uroporphyrin-induced bone defects in congenital erythropoietic porphyria models
Proposed model of CEP pathogenesis. UROS inhibition leads to production of uro/copro-I mostly in erythrocytes and liver, which is transported through blood to the bones. Uro-I causes bone damage by binding to hydroxyapatite, causing oxidative and ER stress, protein aggregation and stalled autophagy. Acitretin partially rescues uro-I-induced bone damage by reducing oxidative and ER stress and restoring autophagic flux.
