Impaired mitophagy contributes to osteogenesis and mineralization disorders in fibrous dysplasia - PubMed
12 hours ago
- #mitophagy
- #fibrous-dysplasia
- #biomineralization
- Fibrous dysplasia (FD) is caused by GNAS gene mutation, leading to excessive fibrous tissue and immature trabecular bone formation.
- Impaired mitophagy due to hyperactivation of the cAMP-PRKA/PKA signaling disrupts mitochondrial homeostasis in FD.
- PRKA activation inhibits DNM1L/DRP1 recruitment, suppressing mitochondrial fission and leading to excessive fusion.
- Elevated mitochondrial membrane potential and impaired mitophagy result in diminished amorphous calcium phosphate (ACP) secretion.
- The study links cAMP-PRKA signaling, mitochondrial dynamics, and biomineralization in FD pathogenesis.