{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:11:17Z","timestamp":1792033877065,"version":"4.4.0"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2023,7,12]],"date-time":"2023-07-12T00:00:00Z","timestamp":1689120000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Association of British Neurologists and Guarantors of Brain"},{"name":"Christos Lazari Foundation"},{"DOI":"10.13039\/100013560","name":"GBS-CIDP Foundation International","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013560","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001543","name":"New Zealand Neurological Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001543","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Human Frontier Science Program Long-Term Fellowship"},{"name":"Wallenberg Scholar"},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"publisher","award":["#2022-01018"],"award-info":[{"award-number":["#2022-01018"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"publisher","award":["#2019-02397"],"award-info":[{"award-number":["#2019-02397"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Union\u2019s Horizon Europe","award":["101053962"],"award-info":[{"award-number":["101053962"]}]},{"name":"Swedish State Support for Clinical Research","award":["#ALFGBG-71320"],"award-info":[{"award-number":["#ALFGBG-71320"]}]},{"name":"UK Dementia Research Institute at UCL","award":["UKDRI-1003"],"award-info":[{"award-number":["UKDRI-1003"]}]},{"DOI":"10.13039\/501100000627","name":"Guarantors of Brain","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000627","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008721","name":"University College London Hospitals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008721","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100014461","name":"Biomedical Research Centre","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100014461","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Valid, responsive blood biomarkers specific to peripheral nerve damage would improve management of peripheral nervous system (PNS) diseases. Neurofilament light chain (NfL) is sensitive for detecting axonal pathology but is not specific to PNS damage, as it is expressed throughout the PNS and CNS. Peripherin, another intermediate filament protein, is almost exclusively expressed in peripheral nerve axons. We postulated that peripherin would be a promising blood biomarker of PNS axonal damage.<\/jats:p>\n               <jats:p>We demonstrated that peripherin is distributed in sciatic nerve, and to a lesser extent spinal cord tissue lysates, but not in brain or extra-neural tissues. In the spinal cord, anti-peripherin antibody bound only to the primary cells of the periphery (anterior horn cells, motor axons and primary afferent sensory axons). In vitro models of antibody-mediated axonal and demyelinating nerve injury showed marked elevation of peripherin levels only in axonal damage and only a minimal rise in demyelination.<\/jats:p>\n               <jats:p>We developed an immunoassay using single molecule array technology for the detection of serum peripherin as a biomarker for PNS axonal damage. We examined longitudinal serum peripherin and NfL concentrations in individuals with Guillain-Barr\u00e9 syndrome (GBS, n = 45, 179 time points), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n = 35, 70 time points), multiple sclerosis (n = 30), dementia (as non-inflammatory CNS controls, n = 30) and healthy individuals (n = 24).<\/jats:p>\n               <jats:p>Peak peripherin levels were higher in GBS than all other groups (median 18.75 pg\/ml versus &amp;lt; 6.98 pg\/ml, P &amp;lt; 0.0001). Peak NfL was highest in GBS (median 220.8 pg\/ml) and lowest in healthy controls (median 5.6 pg\/ml), but NfL did not distinguish between CIDP (17.3 pg\/ml), multiple sclerosis (21.5 pg\/ml) and dementia (29.9 pg\/ml). While peak NfL levels were higher with older age (rho = +0.39, P &amp;lt; 0.0001), peak peripherin levels did not vary with age.<\/jats:p>\n               <jats:p>In GBS, local regression analysis of serial peripherin in the majority of individuals with three or more time points of data (16\/25) displayed a rise-and-fall pattern with the highest value within the first week of initial assessment. Similar analysis of serial NfL concentrations showed a later peak at 16 days. Group analysis of serum peripherin and NfL levels in GBS and CIDP patients were not significantly associated with clinical data, but in some individuals with GBS, peripherin levels appeared to better reflect clinical outcome measure improvement.<\/jats:p>\n               <jats:p>Serum peripherin is a promising new, dynamic and specific biomarker of acute PNS axonal damage.<\/jats:p>","DOI":"10.1093\/brain\/awad234","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T20:05:59Z","timestamp":1689105959000},"page":"4562-4573","source":"Crossref","is-referenced-by-count":83,"title":["Peripherin is a biomarker of axonal damage in peripheral nervous system disease"],"prefix":"10.1093","volume":"146","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2102-6053","authenticated-orcid":false,"given":"Stephen","family":"Keddie","sequence":"first","affiliation":[{"name":"Department of Neuromuscular Diseases, Barts Health NHS Trust , London E1 1BB , UK"},{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"},{"name":"Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Duncan","family":"Smyth","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"},{"name":"Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6434-9496","authenticated-orcid":false,"given":"Ryan Y S","family":"Keh","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"},{"name":"Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3174-6027","authenticated-orcid":false,"given":"Michael K L","family":"Chou","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"},{"name":"NHS Neuroimmunology and CSF Laboratory, Queen Square Institute of Neurology , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donna","family":"Grant","sequence":"additional","affiliation":[{"name":"NHS Neuroimmunology and CSF Laboratory, Queen Square Institute of Neurology , London WC1N 3BG , UK"},{"name":"Department of Neuroinflammation, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunaina","family":"Surana","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Heslegrave","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London , London WC1E 6BT , UK"},{"name":"Department of Neurodegenerative Disease, UCL Institute of Neurology , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Zetterberg","sequence":"additional","affiliation":[{"name":"UK Dementia Research Institute, University College London , London WC1E 6BT , UK"},{"name":"Department of Neurodegenerative Disease, UCL Institute of Neurology , London WC1N 3BG , UK"},{"name":"Hong Kong Center for Neurodegenerative Diseases , Hong Kong , China"},{"name":"Wisconsin Alzheimer\u2019s Disease Research Center, University of Wisconsin School of Medicine and Public Health, University of Wisconsin-Madison , Madison, WI 53792 , USA"},{"name":"Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, The Sahlgrenska Academy at the University of Gothenburg , M\u00f6lndal 431 41 , Sweden"},{"name":"Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital , M\u00f6lndal 431 41 , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2522-4081","authenticated-orcid":false,"given":"Luuk","family":"Wieske","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurophysiology, Amsterdam Neuroscience, Amsterdam UMC, Location AMC, University of Amsterdam , 1081 HV Amsterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Milou","family":"Michael","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurophysiology, Amsterdam Neuroscience, Amsterdam UMC, Location AMC, University of Amsterdam , 1081 HV Amsterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filip","family":"Eftimov","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurophysiology, Amsterdam Neuroscience, Amsterdam UMC, Location AMC, University of Amsterdam , 1081 HV Amsterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Bellanti","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford OX3 9DU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Rinaldi","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford OX3 9DU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie S","family":"Hart","sequence":"additional","affiliation":[{"name":"NHS Neuroimmunology and CSF Laboratory, Queen Square Institute of Neurology , London WC1N 3BG , UK"},{"name":"Department of Neuroinflammation, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0344-9749","authenticated-orcid":false,"given":"Axel","family":"Petzold","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurophysiology, Amsterdam Neuroscience, Amsterdam UMC, Location AMC, University of Amsterdam , 1081 HV Amsterdam , The Netherlands"},{"name":"UCL Clinical and Movement Neurosciences Department, National Hospital for Neurology and Neurosurgery, UCL Institute of Neurology , London WC1E 6BT , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P","family":"Lunn","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London , London WC1N 3BG , UK"},{"name":"Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery , London WC1N 3BG , 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