The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors.
BACKGROUND: Pericentrin (Pcnt), a conserved protein of the pericentriolar material, serves as a multifunctional scaffold for numerous proteins and plays an important role in microtubule organization. Recent studies indicate that Pcnt mutations are associated with a range of diseases including primor...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2011-01-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC3198765?pdf=render |
| _version_ | 1819063017239740416 |
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| author | Johanna Mühlhans Johann Helmut Brandstätter Andreas Giessl |
| author_facet | Johanna Mühlhans Johann Helmut Brandstätter Andreas Giessl |
| author_sort | Johanna Mühlhans |
| collection | DOAJ |
| description | BACKGROUND: Pericentrin (Pcnt), a conserved protein of the pericentriolar material, serves as a multifunctional scaffold for numerous proteins and plays an important role in microtubule organization. Recent studies indicate that Pcnt mutations are associated with a range of diseases including primordial dwarfism and ciliopathies. To date, three Pcnt splice variants from orthologous genes in mice and humans are known. PRINCIPAL FINDINGS: We generated a specific Pcnt antiserum detecting all known Pcnt splice variants and examined the cellular and subcellular distribution of Pcnt in ciliated tissues of the mouse, the olfactory epithelium and the retina. For the first time, we identified Pcnt and its centrosomal interaction partners at the basal body complex of mouse retinal photoreceptors. Photoreceptors are morphologically and functionally subdivided into the light sensitive outer segment and the inner segment comprising the metabolic function of the cell. The two compartments are linked via a modified, specialized, non-motile cilium, the connecting cilium. Here, Pcnt colocalized with the whole protein machinery responsible for transport processes between the two compartments. Surprisingly, photoreceptors expressed a small Pcnt splice transcript - most likely a modified variant of Pcnt S - which was not present in receptor neurons of the olfactory epithelium. CONCLUSIONS: Our findings suggest distinct functional roles of several Pcnt variants in different ciliated tissues and sensory neurons, like the olfactory epithelium and the retina of the mouse. The individual patchwork of different Pcnt splice transcripts seems to reflect the complexity of Pcnt function, an assumption corroborated by the heterogeneous clinical manifestations associated with mutations in the Pcnt gene. |
| first_indexed | 2024-12-21T15:07:59Z |
| format | Article |
| id | doaj.art-6c33792431cc4fc4bed0d9729b71207f |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-21T15:07:59Z |
| publishDate | 2011-01-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-6c33792431cc4fc4bed0d9729b71207f2022-12-21T18:59:22ZengPublic Library of Science (PLoS)PLoS ONE1932-62032011-01-01610e2649610.1371/journal.pone.0026496The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors.Johanna MühlhansJohann Helmut BrandstätterAndreas GiesslBACKGROUND: Pericentrin (Pcnt), a conserved protein of the pericentriolar material, serves as a multifunctional scaffold for numerous proteins and plays an important role in microtubule organization. Recent studies indicate that Pcnt mutations are associated with a range of diseases including primordial dwarfism and ciliopathies. To date, three Pcnt splice variants from orthologous genes in mice and humans are known. PRINCIPAL FINDINGS: We generated a specific Pcnt antiserum detecting all known Pcnt splice variants and examined the cellular and subcellular distribution of Pcnt in ciliated tissues of the mouse, the olfactory epithelium and the retina. For the first time, we identified Pcnt and its centrosomal interaction partners at the basal body complex of mouse retinal photoreceptors. Photoreceptors are morphologically and functionally subdivided into the light sensitive outer segment and the inner segment comprising the metabolic function of the cell. The two compartments are linked via a modified, specialized, non-motile cilium, the connecting cilium. Here, Pcnt colocalized with the whole protein machinery responsible for transport processes between the two compartments. Surprisingly, photoreceptors expressed a small Pcnt splice transcript - most likely a modified variant of Pcnt S - which was not present in receptor neurons of the olfactory epithelium. CONCLUSIONS: Our findings suggest distinct functional roles of several Pcnt variants in different ciliated tissues and sensory neurons, like the olfactory epithelium and the retina of the mouse. The individual patchwork of different Pcnt splice transcripts seems to reflect the complexity of Pcnt function, an assumption corroborated by the heterogeneous clinical manifestations associated with mutations in the Pcnt gene.http://europepmc.org/articles/PMC3198765?pdf=render |
| spellingShingle | Johanna Mühlhans Johann Helmut Brandstätter Andreas Giessl The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. PLoS ONE |
| title | The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. |
| title_full | The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. |
| title_fullStr | The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. |
| title_full_unstemmed | The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. |
| title_short | The centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors. |
| title_sort | centrosomal protein pericentrin identified at the basal body complex of the connecting cilium in mouse photoreceptors |
| url | http://europepmc.org/articles/PMC3198765?pdf=render |
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