Complement profiles in patients with amyotrophic lateral sclerosis: A prospective observational cohort study
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- Complement Profiles in Patients with Amyotrophic Lateral Sclerosis: A Prospective Observational Cohort Study
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Background: The complement system has been suggested to be involved in the pathophy- siology of amyotrophic lateral sclerosis (ALS), a progressive motor neuron disease. In the present study, we compared levels of selected complement markers to clinical outcome in ALS patients. Methods: This observational, explorative cohort study included 92 ALS patients, 61 neurological controls (NCs) admitted for suspected aneurysmal subarachnoid haemorrhage, and 96 neurologically healthy controls (NHCs). Peripheral blood and cerebrospinal fluid (CSF) were obtained for the measurement of ficolin-1, -2, and -3; collectin-11, MBL, MASP-3, MAP-1, C4, C3, PTX-3, and complement activation products C4c, C3bc, and sC5b-9. We recorded clinical outcomes of ALS patients for 24 to 48 months after inclusion in order to analyse the effects of the complement markers on survival time. Results: Compared with both control groups, ALS patients exhibited increased collectin-11, C4 and sC5b-9 in plasma, as well as increased ficolin-3 in CSF. Ficolin-2 was significantly decreased in plasma of the ALS patients compared with NHCs, but not with NCs. The concentration of collectin-11, C3 and C3bc correlated negatively with the revised ALS functional rating scale (ALSFRS-R). No association was found between levels of comple- ment markers and survival as estimated by hazard ratios. Conclusion: ALS patients exhibit aberrant expression of selected mediators of the lectin complement pathway as well as increased activation of the terminal complement pathway, corroborating the notion that the complement system might be involved in the pathophysiol- ogy of ALS.
Original language | English |
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Journal | Journal of Inflammation Research |
Volume | 14 |
Pages (from-to) | 1043-1053 |
Number of pages | 11 |
ISSN | 1178-7031 |
DOIs | |
Publication status | Published - 2021 |
- Amyotrophic lateral sclerosis, Cerebrospinal fluid, Complement, Innate immunity, Lectin pathway, Observational cohort study, Pathophysiology
Research areas
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