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- amyotrophic lateral sclerosis
- motor neuron disease
- frontotemporal dementia
A broad body of evidence supports both pathogenic and neuroprotective roles for inflammation in amyotrophic lateral sclerosis (ALS)1 but trials of several immunomodulatory drugs have not yielded a beneficial effect in an increasingly complex disorder with multiple upstream cellular causes.2 There has been interest in proteins involved in the microglial response as potential biomarkers for well over a decade.3 4 Recently this has focused on a group of three chitinase proteins, thought to be macrophage-derived, identified in proteomic analysis of cerebrospinal fluid (CSF) taken from ALS patients.5 6 Chitinases have also been studied the pathologically related disorder frontotemporal dementia (FTD).7 8 Although chitin is not produced by mammals, it is suggested that chitinases might act on N-acetylglucosamine-containing extracellular matrix polymers, such as hyaluronan or keratan sulfate, …
AGT and MRT contributed equally.
Correction notice This article has been corrected since it was published Online First. The title has changed from "amyotrophiclateral sclerosis" to "amyotrophic lateral sclerosis"
Contributors AGT and MRT drafted and edited the manuscript.
Funding This study has been funded by Motor Neurone Disease Association.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; internally peer reviewed.
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