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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.<\/jats:p>","DOI":"10.7554\/elife.38309","type":"journal-article","created":{"date-parts":[[2018,10,12]],"date-time":"2018-10-12T11:00:14Z","timestamp":1539342014000},"source":"Crossref","is-referenced-by-count":60,"title":["High N-glycan multiplicity is critical for neuronal adhesion and sensitizes the developing cerebellum to N-glycosylation defect"],"prefix":"10.7554","volume":"7","author":[{"given":"Daniel","family":"Medina-Cano","sequence":"first","affiliation":[{"name":"Paris Descartes-Sorbonne Paris Cite\u0301 University, Paris, France"},{"name":"Developmental Brain Disorders Laboratory, Imagine Institute, INSERM UMR 1163, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekin","family":"Ucuncu","sequence":"additional","affiliation":[{"name":"Paris Descartes-Sorbonne Paris Cite\u0301 University, Paris, France"},{"name":"Developmental 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