{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T10:57:11Z","timestamp":1792580231642,"version":"4.5.0"},"reference-count":90,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dement Geriatr Cogn Disord"],"published-print":{"date-parts":[[2020]]},"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Background:&lt;\/i&gt;&lt;\/b&gt; Chronic glial dysfunction may contribute to the pathogenesis of frontotemporal dementia (FTD). Cerebrospinal fluid (CSF) levels of glia-derived proteins YKL-40 and chitotriosidase are increased in Alzheimer\u2019s disease (AD) but have not been explored in detail across the spectrum of FTD. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; We investigated whether CSF YKL-40 and chitotriosidase levels differed between FTD patients and controls, across different clinical and genetic subtypes of FTD, and between individuals with a clinical FTD syndrome due to AD versus non-AD (frontotemporal lobar degeneration, FTLD) pathology (based on CSF neurodegenerative biomarkers). Eighteen healthy controls and 64 people with FTD (behavioural variant FTD, &lt;i&gt;n&lt;\/i&gt; = 20; primary progressive aphasia [PPA], &lt;i&gt;n&lt;\/i&gt; = 44: nfvPPA, &lt;i&gt;n&lt;\/i&gt; = 16, svPPA, &lt;i&gt;n&lt;\/i&gt; = 11, lvPPA, &lt;i&gt;n&lt;\/i&gt; = 14, PPA-NOS, &lt;i&gt;n&lt;\/i&gt; = 3) were included. 10\/64 had familial FTD, with mutations in&lt;i&gt; GRN&lt;\/i&gt;(&lt;i&gt;n&lt;\/i&gt; = 3), &lt;i&gt;MAPT&lt;\/i&gt;(&lt;i&gt;n&lt;\/i&gt; = 4), or &lt;i&gt;C9orf72&lt;\/i&gt; (&lt;i&gt;n&lt;\/i&gt; = 3). 15\/64 had neurodegenerative biomarkers consistent with AD pathology. Levels were measured by immunoassay and compared using multiple linear regressions. We also examined relationships of YKL-40 and chitotriosidase with CSF total tau (T-tau), phosphorylated tau 181 (P-tau) and \u03b2-amyloid 1\u201342 (A\u03b242), with each other, and with age and disease du\u00adration. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; CSF YKL-40 and chitotriosidase levels were higher in FTD, particularly lvPPA (both) and nfvPPA (YKL-40), compared with controls. &lt;i&gt;GRN&lt;\/i&gt; mutation carriers had higher levels of both proteins than controls and &lt;i&gt;C9orf72&lt;\/i&gt; expansion carriers, and YKL-40 was higher in &lt;i&gt;MAPT&lt;\/i&gt; mutation carriers than controls. Individuals with underlying AD pathology had higher YKL-40 and chitotriosidase levels than both controls and those with likely FTLD pathology. CSF YKL-40 and chitotriosidase levels were variably associated with levels of T-tau, P-tau and A\u03b242, and with each other, depending on clinical syndrome and underlying pathology. CSF YKL-40 but not chitotriosidase was associated with age, but not disease duration. &lt;b&gt;&lt;i&gt;Conclusion:&lt;\/i&gt;&lt;\/b&gt; CSF YKL-40 and chitotriosidase levels are increased in individuals with clinical FTD syndromes, particularly due to AD pathology. In a preliminary analysis of genetic groups, levels of both proteins are found to be highly elevated in FTD due to&lt;i&gt; GRN&lt;\/i&gt; mutations, while YKL-40 is increased in individuals with &lt;i&gt;MAPT&lt;\/i&gt; mutations. As glia-derived protein levels generally correlate with T-tau and P-tau levels, they may reflect the glial response to neurodegeneration in FTLD.<\/jats:p>","DOI":"10.1159\/000506282","type":"journal-article","created":{"date-parts":[[2020,4,28]],"date-time":"2020-04-28T17:00:28Z","timestamp":1588093228000},"page":"56-76","source":"Crossref","is-referenced-by-count":47,"title":["Cerebrospinal Fluid YKL-40 and Chitotriosidase Levels in Frontotemporal Dementia Vary by Clinical, Genetic and Pathological 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