Title40 Years of CSF Toxicity Studies in ALS: What Have We Learnt About ALS Pathophysiology?
Publication TypeJournal Article
Year of Publication2021
AuthorsKwong KChong Ng K, Harbham PK, Selvaraj BT, Gregory JM, Pal S, Hardingham GE, Chandran S, Mehta AR
JournalFront Mol Neurosci
Date Published2021

Based on early evidence of neurotoxicity following exposure to serum derived from patients with amyotrophic lateral sclerosis (ALS), several studies have attempted to explore whether cerebrospinal fluid (CSF) obtained from people with ALS could possess similar properties. Although initial findings proved inconclusive, it is now increasingly recognized that ALS-CSF may exert toxicity both and . Nevertheless, the mechanism underlying CSF-induced neurodegeneration remains unclear. This review aims to summarize the 40-year long history of CSF toxicity studies in ALS, while discussing the various mechanisms that have been proposed, including glutamate excitotoxicity, proteotoxicity and oxidative stress. Furthermore, we consider the potential implications of a toxic CSF circulatory system in the pathophysiology of ALS, and also assess its significance in the context of current ALS research.

Alternate JournalFront Mol Neurosci
PubMed ID33815058
PubMed Central IDPMC8012723
Grant ListMEHTA/JUL17/948-795 / MNDA_ / Motor Neurone Disease Association / United Kingdom
MR/L023784/2 / MRC_ / Medical Research Council / United Kingdom
MR/R001162/1 / MRC_ / Medical Research Council / United Kingdom