The centrosomal/basal body protein OFD1 is required for microtubule organization and cell cycle progression
| Autor: | Gaetano Calì, Gerard W. Dougherty, Lucio Nitsch, Lucia Perone, Brunella Franco, Daniela Iaconis, Roberta Tammaro, Antonella Ragnini-Wilson, Mariaevelina Alfieri |
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| Přispěvatelé: | Alfieri, M., Iaconis, D., Tammaro, R., Perone, L., Cali, G., Nitsch, L., Dougherty, G. W., Ragnini, A., Franco, B. |
| Rok vydání: | 2019 |
| Předmět: |
Cell type
Cell division Primary Cell Culture Biology Cell Line 03 medical and health sciences Microtubule Basal body Animals Humans Cilia Settore BIO/10 Cell Nucleu Orofaciodigital Syndrome Cytoskeleton 030304 developmental biology Cell Nucleus Centrosome 0303 health sciences Cell growth Animal 030305 genetics & heredity Cell Cycle Proteins Microtubule organizing center Cell Biology General Medicine Cell cycle Basal Bodie Fibroblasts Orofaciodigital Syndromes Aneuploidy Basal Bodies Cell biology Mutation OFD1 Microtubule-Organizing Center Fibroblast Developmental Biology Human |
| Zdroj: | Tissuecell. 64 |
| ISSN: | 1532-3072 |
| Popis: | Oral-Facial-Digital type I (OFD1) is a rare inherited form of renal cystic disease associated with ciliary dysfunction. This disorder is due to mutations in the OFD1 gene that encodes a protein localized to centrosomes and basal bodies in different cell types. Immunofluorescence analysis demonstrated that OFD1 displays a dynamic distribution during cell cycle. High-content microscopy analysis of Ofd1-depleted fibroblasts revealed impaired cell cycle progression. Immunofluorescence analysis and cell proliferation assays also indicated the presence of a variety of defects such as centrosome accumulation, nuclear abnormalities and aneuploidy. In addition, Ofd1-depleted cells displayed an abnormal microtubule network that may underlie these defects. All together our results suggest that OFD1 contributes to the function of the microtubule organizing center (MTOC) in the cell, controlling cell cycle progression both in vitro and in vivo. |
| Databáze: | OpenAIRE |
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