2022
DOI: 10.1002/acn3.51656
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Chitotriosidase is a biomarker for adult‐onset leukoencephalopathy with axonal spheroids and pigmented glia

Abstract: Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) leads to rapidly progressive dementia and is caused by mutations in the gene CSF1R. Neurodegeneration is driven by dysfunction of microglia, the predominant cell type expressing CSF1R in the brain. We assessed chitotriosidase, an enzyme secreted by microglia, in serum and cerebrospinal fluid of patients with ALSP. Chitotriosidase activity was highly increased in cerebrospinal fluid of patients and correlated inversely with disease … Show more

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Cited by 3 publications
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“…To better understand the molecular changes in the leukodystrophies group, which are primarily characterized by cellular dysfunction at the level of glial cells, it is essential to assess and characterize biomarkers related to the main pathological process, as exemplified by the analysis of glial fibrillary acidic protein (GFAP) as a biomarker of astrocytic dysfunction in X-linked adrenoleukodystrophy, metachromatic leukodystrophy, and Alexander disease [52][53][54]. Additionally, in the context of ALSP, microglial activity markers such as chitinases may serve as promising biomarkers to explore, as demonstrated by our results and as previously reported by one study in the literature [55].…”
Section: Discussionsupporting
confidence: 63%
“…To better understand the molecular changes in the leukodystrophies group, which are primarily characterized by cellular dysfunction at the level of glial cells, it is essential to assess and characterize biomarkers related to the main pathological process, as exemplified by the analysis of glial fibrillary acidic protein (GFAP) as a biomarker of astrocytic dysfunction in X-linked adrenoleukodystrophy, metachromatic leukodystrophy, and Alexander disease [52][53][54]. Additionally, in the context of ALSP, microglial activity markers such as chitinases may serve as promising biomarkers to explore, as demonstrated by our results and as previously reported by one study in the literature [55].…”
Section: Discussionsupporting
confidence: 63%