2018
DOI: 10.18632/aging.101551
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Abstract: Amyotrophic lateral sclerosis (ALS) has variable clinical course and fatal outcome. Since inflammation plays a role in the pathogenesis of ALS, chitinase-3-like protein 1 or YKL40 has been assessed as putative biomarker of disease progression. YKL40 mRNA levels are increased in anterior horn of the spinal cord (P=0.004) in sporadic ALS (sALS) cases when compared with age-matched controls. These correlate with increased mRNA expression of microglial markers AIF1 and CD68 in the spinal cord in sALS (P=0.044 and … Show more

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Cited by 17 publications
(25 citation statements)
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References 87 publications
(41 reference statements)
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“…We found CHI3L1 levels to increase with age, as also demonstrated for ALS, 46,49 and to be associated with antibody-related CSF measures such as OCB-positive status and IgG index but most strongly the number of OCBs. CHI3L1 levels have been reported as a predictor for the conversion from clinically isolated syndrome to MS, although not consistently for longer follow-up times or for radiologically isolated syndrome.…”
Section: Discussionsupporting
confidence: 78%
“…We found CHI3L1 levels to increase with age, as also demonstrated for ALS, 46,49 and to be associated with antibody-related CSF measures such as OCB-positive status and IgG index but most strongly the number of OCBs. CHI3L1 levels have been reported as a predictor for the conversion from clinically isolated syndrome to MS, although not consistently for longer follow-up times or for radiologically isolated syndrome.…”
Section: Discussionsupporting
confidence: 78%
“…Elevated levels of Chit-1 and CHI3L1 in the CSF are indicative of glial activation that may potentiate neuroinflammation and motor neuron death in ALS. Prior studies demonstrated increased astrocyte infiltration in the spinal cord of Superoxide dismutase 1 ( SOD1 ) transgenic mice,34 and the presence of CHI3L1-positive cells in the lumbar spinal cord of patients with ALS 35. Positron emission tomography demonstrated increased numbers of activated microglia in the motor cortex and corticospinal tract in patients with ALS 36.…”
Section: Discussionmentioning
confidence: 99%
“…Different clinical subtypes of FTD may have widely differing pathologies, co-pathologies and disease mechanisms and hence differing degrees of glial activation which could affect YKL-40 and chitotriosidase release. In particular, patients with MND have much higher CSF YKL-40 levels [46,48,[52][53][54][55] and CSF chitotriosidase levels or activity [34,48,51,[54][55][56][57] than controls, and higher chitotriosidase levels than in FTD [51], so our study did not include individuals with FTD-MND to avoid confounding results. The majority (78%) of our lvPPA subgroup had a CSF biomarker profile consistent with AD (rather than FTLD), and our nfvPPA subgroup contained 2 individuals with AD-like CSF biomarkers and 2 individuals with GRN mutations.…”
Section: Discussionmentioning
confidence: 99%