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In peripheral nerves, Schwann cells (SCs) myelinate axons and the miRNA biogenesis pathway is required for developmental myelination and myelin maintenance. However, regulatory roles of this pathway at different stages of myelination are only partially understood. We addressed the requirement of the core miRNA biogenesis pathway components Dgcr8, Drosha, and Dicer in developing and adult SCs using mouse mutants with a comparative genetics and transcriptomics approach. We found that the microprocessor components Dgcr8 and Drosha are crucial for axonal radial sorting and to establish correct SC numbers upon myelination. Transcriptome analyses revealed a requirement of the microprocessor to prevent aberrantly increased expression of injury\u2010response genes. Those genes are predicted targets of abundant miRNAs in sciatic nerves (SNs) during developmental myelination. In agreement, Dgcr8 and Dicer are required for proper maintenance of the myelinated SC state, where abundant miRNAs in adult SNs are predicted to target injury\u2010response genes. We conclude that the miRNA biogenesis pathway in SCs is crucial for preventing inappropriate activity of injury\u2010response genes in developing and adult SCs.<\/jats:p>","DOI":"10.1002\/glia.23516","type":"journal-article","created":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T12:54:17Z","timestamp":1539003257000},"page":"2632-2644","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["The miRNA biogenesis pathway prevents inappropriate expression of injury response genes in developing and adult Schwann cells"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3038-4071","authenticated-orcid":false,"given":"Deniz","family":"G\u00f6kbuget","sequence":"first","affiliation":[{"name":"ETH Zurich, Department of Biology Institute of Molecular Health Sciences  Zurich Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jorge 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