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Different neuroinfl...
Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase
- Article/chapterEnglish2019
Publisher, publication year, extent ...
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2018-09-17
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BMJ Publishing Group Ltd,2019
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printrdacarrier
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LIBRIS-ID:oai:DiVA.org:umu-157233
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https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-157233URI
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https://doi.org/10.1136/jnnp-2018-318868DOI
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Language:English
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Summary in:English
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
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Objective: To investigate the role of neuroinflammation in asymptomatic and symptomatic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mutation carriers.Methods: The neuroinflammatory markers chitotriosidase 1 (CHIT1), YKL-40 and glial fibrillary acidic protein (GFAP) were measured in cerebrospinal fluid (CSF) and blood samples from asymptomatic and symptomatic ALS/FTD mutation carriers, sporadic cases and controls by ELISA.Results: CSF levels of CHIT1, YKL-40 and GFAP were unaffected in asymptomatic mutation carriers (n=16). CHIT1 and YKL-40 were increased in gALS (p<0.001, n=65) whereas GFAP was not affected. Patients with ALS carrying a CHIT1 polymorphism had lower CHIT1 concentrations in CSF (-80%) whereas this polymorphism had no influence on disease severity. In gFTD (n=23), increased YKL-40 and GFAP were observed (p<0.05), whereas CHIT1 was nearly not affected. The same profile as in gALS and gFTD was observed in sALS (n=64/70) and sFTD (n=20/26). CSF and blood concentrations correlated moderately (CHIT1, r=0.51) to weak (YKL-40, r=0.30, GFAP, r=0.39). Blood concentrations of these three markers were not significantly altered in any of the groups except CHIT1 in gALS of the Ulm cohort (p<0.05).Conclusion: Our data indicate that neuroinflammation is linked to the symptomatic phase of ALS/FTD and shows a similar pattern in sporadic and genetic cases. ALS and FTD are characterised by a different neuroinflammatory profile, which might be one driver of the diverse presentations of the ALS/FTD syndrome.
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Weydt, Patrick
(author)
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Steinacker, Petra
(author)
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Anderl-Straub, Sarah
(author)
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Nordin, FridaUmeå universitet,Klinisk neurovetenskap(Swepub:umu)franon02
(author)
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Volk, Alexander E.
(author)
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Diehl-Schmid, Janine
(author)
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Andersen, Peter M.,1962-Umeå universitet,Klinisk neurovetenskap,Department of Neurology, Ulm University hospital, Ulm, Germany(Swepub:umu)pean0001
(author)
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Kornhuber, Johannes
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Danek, Adrian
(author)
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Fassbender, Klaus
(author)
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Fliessbach, Klaus
(author)
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Jahn, Holger
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Lauer, Martin
(author)
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Mueller, Kathrin
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Knehr, Antje
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Prudlo, Johannes
(author)
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Schneider, Anja
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Thal, Dietmar R.
(author)
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Yilmazer-Hanke, Deniz
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Weishaupt, Jochen H.
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Ludolph, Albert C.
(author)
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Otto, Markus
(author)
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Umeå universitetKlinisk neurovetenskap
(creator_code:org_t)
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In:Journal of Neurology, Neurosurgery and Psychiatry: BMJ Publishing Group Ltd90:1, s. 4-100022-30501468-330X
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Oeckl, Patrick
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Weydt, Patrick
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Steinacker, Petr ...
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Anderl-Straub, S ...
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Nordin, Frida
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Volk, Alexander ...
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Diehl-Schmid, Ja ...
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Andersen, Peter ...
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Kornhuber, Johan ...
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Danek, Adrian
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Fassbender, Klau ...
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Fliessbach, Klau ...
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Jahn, Holger
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Lauer, Martin
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Mueller, Kathrin
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Knehr, Antje
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Prudlo, Johannes
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Schneider, Anja
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Thal, Dietmar R.
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Yilmazer-Hanke, ...
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Weishaupt, Joche ...
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Ludolph, Albert ...
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Otto, Markus
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Umeå University