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| Status |
Public on Dec 31, 2022 |
| Title |
Cystic fibrosis causes olfactory system defects by altering progenitor cell proliferation (scRNA-Seq) |
| Organism |
Sus scrofa |
| Experiment type |
Expression profiling by high throughput sequencing
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| Summary |
A reduced sense of smell has been reported in people with cystic fibrosis (CF). These olfactory defects have largely been attributed to secondary manifestations of the disease, such as inflammation of the nasal mucosa. Here we show that CFTR, the gene responsible for CF, is expressed in proliferating olfactory human cells and that newborn CFTR null pigs display ultrastructural abnormalities in the olfactory epithelium and olfactory bulbs. In the absence of CFTR, olfactory sensory neurons still produce odor-evoked activity, but mutant animals display defective odor-guided suckling behavior after birth. Consistent with epithelial changes, we found a reduced expression of genes implicated in cell cycle and development in globose basal cells (GBCs), the neurogenic progenitor cells in the olfactory epithelium. Targeted sequencing revealed enhanced CFTR expression in the subpopulation of GBCs that is actively proliferating. Furthermore, CFTR loss caused a global reduction in the number of sensory neurons and altered olfactory receptors expression. Our findings highlight a previously unknown role of CFTR in olfactory system function by regulating progenitor cell proliferation in the olfactory epithelium.
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| Overall design |
The molecular pathology of olfactory loss in CF.
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| Contributor(s) |
Caballero I, López-Gálvez R, Mbouamboua Y, Couralet M, Fleurot I, Pons N, Barrera-Conde M, Quílez-Playán N, Keller M, Klymiuk N, Robledo P, Barbry P, Chamero P |
| Citation missing |
Has this study been published? Please login to update or notify GEO. |
| |
| Submission date |
Feb 22, 2022 |
| Last update date |
Dec 31, 2022 |
| Contact name |
Kevin Lebrigand |
| Organization name |
IPMC/CNRS
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| Lab |
Functional Genomics Platform of Nice-Sophia-Antipolis, France.
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| Street address |
660 route des lucioles
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| City |
Valbonne - Sophia-Antipolis |
| ZIP/Postal code |
06560 |
| Country |
France |
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| Platforms (1) |
| GPL20983 |
Illumina NextSeq 500 (Sus scrofa) |
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| Samples (2) |
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| This SubSeries is part of SuperSeries: |
| GSE197187 |
Cystic fibrosis causes olfactory system defects by altering progenitor cell proliferation |
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| Relations |
| BioProject |
PRJNA809339 |
