{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T00:08:29Z","timestamp":1790122109995,"version":"4.0.1"},"reference-count":48,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2021,7,5]],"date-time":"2021-07-05T00:00:00Z","timestamp":1625443200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Funda\u00e7\u00e3o para a Ci\u00eancia e Tecnologia\/Minist\u00e9rio da Ci\u00eancia, Tecnologia e Ensino Superior","award":["iNOVA4Health \u2013 UIDB\/04462\/2020 and UIDP\/04462\/2020"],"award-info":[{"award-number":["iNOVA4Health \u2013 UIDB\/04462\/2020 and UIDP\/04462\/2020"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Amyotrophic lateral sclerosis (ALS) is a neurodegenerative neuromuscular disease that affects motor neurons controlling voluntary muscles. Survival is usually 2\u20135 years after onset, and death occurs due to respiratory failure. The identification of biomarkers would be very useful to help in disease diagnosis and for patient stratification based on, e.g., progression rate, with implications in therapeutic trials. Neurofilaments constitute already-promising markers for ALS and, recently, chitinases have emerged as novel marker targets for the disease. Here, we investigated cerebrospinal fluid (CSF) chitinases as potential markers for ALS. Chitotriosidase (CHIT1), chitinase-3-like protein 1 (CHI3L1), chitinase-3-like protein 2 (CHI3L2) and the benchmark marker phosphoneurofilament heavy chain (pNFH) were quantified by an enzyme-linked immunosorbent assay (ELISA) from the CSF of 34 ALS patients and 24 control patients with other neurological diseases. CSF was also analyzed by UHPLC-mass spectrometry. All three chitinases, as well as pNFH, were found to correlate with disease progression rate. Furthermore, CHIT1 was elevated in ALS patients with high diagnostic performance, as was pNFH. On the other hand, CHIT1 correlated with forced vital capacity (FVC). The three chitinases correlated with pNFH, indicating a relation between degeneration and neuroinflammation. In conclusion, our results supported the value of CHIT1 as a diagnostic and progression rate biomarker, and its potential as respiratory function marker. The results opened novel perspectives to explore chitinases as biomarkers and their functional relevance in ALS.<\/jats:p>","DOI":"10.3390\/diagnostics11071210","type":"journal-article","created":{"date-parts":[[2021,7,5]],"date-time":"2021-07-05T04:18:21Z","timestamp":1625458701000},"page":"1210","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Cerebrospinal Fluid Chitinases as Biomarkers for Amyotrophic Lateral Sclerosis"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7782-6319","authenticated-orcid":false,"given":"J\u00falia","family":"Costa","sequence":"first","affiliation":[{"name":"Instituto de Tecnologia Qu\u00edmica e Biol\u00f3gica Ant\u00f3nio Xavier, Universidade Nova de Lisboa, Avenida da Rep\u00fablica, 2780-157 Oeiras, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2111-4579","authenticated-orcid":false,"given":"Marta","family":"Gromicho","sequence":"additional","affiliation":[{"name":"Instituto de Fisiologia, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Pronto-Laborinho","sequence":"additional","affiliation":[{"name":"Instituto de Fisiologia, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Concei\u00e7\u00e3o","family":"Almeida","sequence":"additional","affiliation":[{"name":"UniMS\u2014Mass Spectrometry Unit, iBET\u2014Instituto de Biologia Experimental e Tecnologica, 2780-157 Oeiras, Portugal"},{"name":"UniMS\u2014Mass Spectrometry Unit, ITQB\u2014Instituto de Tecnologia Quimica e Biologica Antonio Xavier, Universidade Nova de Lisboa, 2780-157 Oeiras, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo A.","family":"Gomes","sequence":"additional","affiliation":[{"name":"UniMS\u2014Mass Spectrometry Unit, iBET\u2014Instituto de Biologia Experimental e Tecnologica, 2780-157 Oeiras, Portugal"},{"name":"UniMS\u2014Mass Spectrometry Unit, ITQB\u2014Instituto de Tecnologia Quimica e Biologica Antonio Xavier, Universidade Nova de Lisboa, 2780-157 Oeiras, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7733-6336","authenticated-orcid":false,"given":"Ana C. L.","family":"Guerreiro","sequence":"additional","affiliation":[{"name":"UniMS\u2014Mass Spectrometry Unit, iBET\u2014Instituto de Biologia Experimental e Tecnologica, 2780-157 Oeiras, Portugal"},{"name":"UniMS\u2014Mass Spectrometry Unit, ITQB\u2014Instituto de Tecnologia Quimica e Biologica Antonio Xavier, Universidade Nova de Lisboa, 2780-157 Oeiras, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8320-0937","authenticated-orcid":false,"given":"Abel","family":"Oliva","sequence":"additional","affiliation":[{"name":"Instituto de Tecnologia Qu\u00edmica e Biol\u00f3gica Ant\u00f3nio Xavier, Universidade Nova de Lisboa, Avenida da Rep\u00fablica, 2780-157 Oeiras, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Pinto","sequence":"additional","affiliation":[{"name":"Instituto de Fisiologia, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mamede","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Instituto de Fisiologia, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Department of Neurosciences and Mental Health, Hospital de Santa Maria-CHULN, 1649-035 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,7,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1001\/jamaneurol.2019.2044","article-title":"Lifetime Risk and Heritability of Amyotrophic Lateral Sclerosis","volume":"76","author":"Ryan","year":"2019","journal-title":"JAMA Neurol."},{"key":"ref_2","first-page":"252","article-title":"Current knowledge and recent insights into the genetic basis of amyotrophic lateral sclerosis","volume":"30","author":"Volk","year":"2018","journal-title":"Med. 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Macromol."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/11\/7\/1210\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:25:58Z","timestamp":1760163958000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/11\/7\/1210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,5]]},"references-count":48,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2021,7]]}},"alternative-id":["diagnostics11071210"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics11071210","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,5]]}}}