High N-glycan multiplicity is critical for neuronal adhesion and sensitizes the developing cerebellum to N-glycosylation defect
Proper brain development relies highly on protein N-glycosylation to sustain neuronal migration, axon guidance and synaptic physiology. Impairing the N-glycosylation pathway at early steps produces broad neurological symptoms identified in congenital disorders of glycosylation. However, little is kn...
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| Hauptverfasser: | , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
12 October 2018
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| In: |
eLife
Year: 2018, Jahrgang: 7 |
| ISSN: | 2050-084X |
| DOI: | 10.7554/eLife.38309 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.7554/eLife.38309
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| Verfasserangaben: | Daniel Medina-Cano, Ekin Ucuncu, Lam Son Nguyen, Michael Nicouleau, Joanna Lipecka, Jean-Charles Bizot, Christian Thiel, François Foulquier, Nathalie Lefort, Catherine Faivre-Sarrailh, Laurence Colleaux, Ida Chiara Guerrera, Vincent Cantagrel |
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| 245 | 1 | 0 | |a High N-glycan multiplicity is critical for neuronal adhesion and sensitizes the developing cerebellum to N-glycosylation defect |c Daniel Medina-Cano, Ekin Ucuncu, Lam Son Nguyen, Michael Nicouleau, Joanna Lipecka, Jean-Charles Bizot, Christian Thiel, François Foulquier, Nathalie Lefort, Catherine Faivre-Sarrailh, Laurence Colleaux, Ida Chiara Guerrera, Vincent Cantagrel |
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| 520 | |a Proper brain development relies highly on protein N-glycosylation to sustain neuronal migration, axon guidance and synaptic physiology. Impairing the N-glycosylation pathway at early steps produces broad neurological symptoms identified in congenital disorders of glycosylation. However, little is known about the molecular mechanisms underlying these defects. We generated a cerebellum specific knockout mouse for Srd5a3, a gene involved in the initiation of N-glycosylation. In addition to motor coordination defects and abnormal granule cell development, Srd5a3 deletion causes mild N-glycosylation impairment without significantly altering ER homeostasis. Using proteomic approaches, we identified that Srd5a3 loss affects a subset of glycoproteins with high N-glycans multiplicity per protein and decreased protein abundance or N-glycosylation level. As IgSF-CAM adhesion proteins are critical for neuron adhesion and highly N-glycosylated, we observed impaired IgSF-CAM-mediated neurite outgrowth and axon guidance in Srd5a3 mutant cerebellum. Our results link high N-glycan multiplicity to fine-tuned neural cell adhesion during mammalian brain development. | ||
| 650 | 4 | |a 3-Oxo-5-alpha-Steroid 4-Dehydrogenase | |
| 650 | 4 | |a Animals | |
| 650 | 4 | |a Axon Guidance | |
| 650 | 4 | |a cell adhesion | |
| 650 | 4 | |a Cell Adhesion | |
| 650 | 4 | |a Cell Adhesion Molecules | |
| 650 | 4 | |a cell biology | |
| 650 | 4 | |a Cell Differentiation | |
| 650 | 4 | |a Cell Membrane | |
| 650 | 4 | |a cerebellum | |
| 650 | 4 | |a Cerebellum | |
| 650 | 4 | |a congenital disorders of glycosylation | |
| 650 | 4 | |a Cytoplasmic Granules | |
| 650 | 4 | |a Gene Deletion | |
| 650 | 4 | |a Glycosylation | |
| 650 | 4 | |a human | |
| 650 | 4 | |a Immunoglobulins | |
| 650 | 4 | |a Induced Pluripotent Stem Cells | |
| 650 | 4 | |a Membrane Proteins | |
| 650 | 4 | |a Mice, Knockout | |
| 650 | 4 | |a Motor Activity | |
| 650 | 4 | |a mouse | |
| 650 | 4 | |a Mutation | |
| 650 | 4 | |a N-glycosylation | |
| 650 | 4 | |a Neural Pathways | |
| 650 | 4 | |a neuronal migration | |
| 650 | 4 | |a Neurons | |
| 650 | 4 | |a neuroscience | |
| 650 | 4 | |a Polysaccharides | |
| 650 | 4 | |a proteomics | |
| 650 | 4 | |a Proteomics | |
| 650 | 4 | |a Purkinje Cells | |
| 650 | 4 | |a Reproducibility of Results | |
| 650 | 4 | |a Unfolded Protein Response | |
| 700 | 1 | |a Ucuncu, Ekin |e VerfasserIn |4 aut | |
| 700 | 1 | |a Nguyen, Lam Son |e VerfasserIn |4 aut | |
| 700 | 1 | |a Nicouleau, Michael |e VerfasserIn |4 aut | |
| 700 | 1 | |a Lipecka, Joanna |e VerfasserIn |4 aut | |
| 700 | 1 | |a Bizot, Jean-Charles |e VerfasserIn |4 aut | |
| 700 | 1 | |a Thiel, Christian |d 1972- |e VerfasserIn |0 (DE-588)124888127 |0 (DE-627)367435276 |0 (DE-576)293543151 |4 aut | |
| 700 | 1 | |a Foulquier, François |e VerfasserIn |4 aut | |
| 700 | 1 | |a Lefort, Nathalie |e VerfasserIn |4 aut | |
| 700 | 1 | |a Faivre-Sarrailh, Catherine |e VerfasserIn |4 aut | |
| 700 | 1 | |a Colleaux, Laurence |e VerfasserIn |4 aut | |
| 700 | 1 | |a Guerrera, Ida Chiara |e VerfasserIn |4 aut | |
| 700 | 1 | |a Cantagrel, Vincent |e VerfasserIn |4 aut | |
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