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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, …
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