{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:36:38Z","timestamp":1792269398566,"version":"4.4.0"},"reference-count":150,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T00:00:00Z","timestamp":1743724800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Ministry of University and Research (MUR), National Recovery and Resilience Plan (NRRP)","award":["PE0000006"],"award-info":[{"award-number":["PE0000006"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Pathological changes and the cellular and molecular mechanisms underlying axonopathy and myelinopathy are key to understanding a wide range of inherited and acquired peripheral nerve disorders. While the clinical indications for nerve biopsy have diminished over time, its diagnostic value remains significant in select conditions, offering a unique window into the pathophysiological processes of peripheral neuropathies. Evidence highlights the symbiotic relationship between axons and myelinating Schwann cells, wherein disruptions in axo-glial interactions contribute to neuropathogenesis. This review synthesizes recent insights into the pathological and molecular underpinnings of axonopathy and myelinopathy. Axonopathy encompasses Wallerian degeneration, axonal atrophy, and dystrophy. Although extensively studied in traumatic nerve injury, the mechanisms of axonal degeneration and Schwann cell-mediated repair are increasingly recognized as pivotal in non-traumatic disorders, including dying-back neuropathies. We briefly outline key transcription factors, signaling pathways, and epigenetic changes driving axonal regeneration. For myelinopathy, we discuss primary segmental demyelination and dysmyelination, characterized by defective myelin development. We describe paranodal demyelination in light of recent findings in nodopathies, emphasizing that it is not an exclusive indicator of demyelinating disorders. This comprehensive review provides a framework to enhance our understanding of peripheral nerve pathology and its implications for developing targeted therapies.<\/jats:p>","DOI":"10.3390\/ijms26073377","type":"journal-article","created":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T06:52:56Z","timestamp":1743749576000},"page":"3377","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Basic Pathological Mechanisms in Peripheral Nerve Diseases"],"prefix":"10.3390","volume":"26","author":[{"given":"Angelo","family":"Schenone","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genoa, Largo P. Daneo 3, 16132 Genova, Italy"},{"name":"IRCCS Ospedale Policlinico San Martino, UO Clinica Neurologica, Largo R. Benzi 10, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-9938-8163","authenticated-orcid":false,"given":"Sara","family":"Massucco","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genoa, Largo P. Daneo 3, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-0442-3549","authenticated-orcid":false,"given":"Cristina","family":"Schenone","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genoa, Largo P. Daneo 3, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Consuelo Barbara","family":"Venturi","sequence":"additional","affiliation":[{"name":"IRCCS Ospedale Policlinico San Martino, UO Patologia, Largo R. Benzi 10, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Nozza","sequence":"additional","affiliation":[{"name":"IRCCS Ospedale Policlinico San Martino, UO Patologia, Largo R. Benzi 10, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5756-0576","authenticated-orcid":false,"given":"Valeria","family":"Prada","sequence":"additional","affiliation":[{"name":"Italian Multiple Sclerosis Foundation (FISM), Scientific Research Area, Via Operai 40, 16149 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-7240-5376","authenticated-orcid":false,"given":"Tania","family":"Pomili","sequence":"additional","affiliation":[{"name":"Electron Microscopy Facility, Istituto Italiano di Tecnologia, Via Morego 30, 16163 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene","family":"Di Patrizi","sequence":"additional","affiliation":[{"name":"IRCCS Ospedale Policlinico San Martino, UO Radiologia, Largo R. 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Benzi 10, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6637-4425","authenticated-orcid":false,"given":"Marina","family":"Grandis","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal and Infantile Sciences (DINOGMI), University of Genoa, Largo P. Daneo 3, 16132 Genova, Italy"},{"name":"IRCCS Ospedale Policlinico San Martino, UO Clinica Neurologica, Largo R. Benzi 10, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1016\/j.mayocp.2015.05.004","article-title":"Peripheral Neuropathy: A Practical Approach to Diagnosis and Symptom Management","volume":"90","author":"Watson","year":"2015","journal-title":"Mayo Clin. 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