The role of OFD1 in selective autophagy

Abstract

Autophagy is a cellular self-degradative pathway. Our study unveiled a novel mechanism mediated by OFD1, the protein mutated in Oral-Facial-Digital type I syndrome, based on selective degradation of autophagic proteins, which enables cells to calibrate their self-degradation. We demonstrated that unrestrained autophagy contributes to renal cysts observed in Ofd1 mutants.

Keywords

• ofd1
• selective autophagy
• autophagy receptor
• oral-facial-digital type i syndrome
• renal cystic disease