Myosin VI regulates ciliogenesis by promoting the turnover of the centrosomal/satellite protein OFD1

ABSTRACT: The actin motor protein myosin VI is a multivalent protein with diverse functions. Here, we identified and characterised a myosin VI ubiquitous interactor, the oral-facial-digital syndrome 1 (OFD1) protein, whose mutations cause malformations of the face, oral cavity, digits, and polycystic kidney disease. We found that myosin VI regulates the localisation of OFD1 at the centrioles and, as a consequence, the recruitment of the distal appendage protein Cep164. Myosin VI depletion in non-tumoral cell lines causes an aberrant localisation of OFD1 along the centriolar walls, which is due to a reduction in the OFD1 mobile fraction. Finally, loss of myosin VI triggers a severe defect in ciliogenesis that could be, at least partially ascribed, to an impairment in the autophagic removal of OFD1 fr

SUBMITTER: Dr. Elisa Magistrati 

PROVIDER: S-SCDT-EMBOR-2021-54160V1 | biostudies-other |

REPOSITORIES: biostudies-other