FIGURE
Figure 4
- ID
- ZDB-FIG-210510-4
- Publication
- Bragazzi Cunha et al., 2021 - Acitretin mitigates uroporphyrin-induced bone defects in congenital erythropoietic porphyria models
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Figure 4
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. |
Expression Data
Expression Detail
Antibody Labeling
Phenotype Data
Phenotype Detail
Acknowledgments
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