Circulating Cytokines Could Not Be Good Prognostic Biomarkers in a Mouse Model of Amyotrophic Lateral Sclerosis
Conclusions: We demonstrated in the SOD1G93A model of ALS that increased levels of several cytokines were associated with a shorter lifespan. However, their role as prognostic biomarkers is unclear as their expression was very variable depending on both the disease stage and the subject. Nevertheless, cytokines may be potential therapeutic targets.
Introduction
Amyotrophic Lateral Sclerosis (ALS) is one of the most common rare diseases of unknown origin that leads to progressive motor neuron degeneration and muscle denervation (1). In particular, it has been described that either distal axonopathy or neuromuscular junction damage precedes motor neuron loss (2). The known mutations that produce the typical adult ALS phenotype are, in order of frequency, the hexanucleotide repeat expansion in C9ORF72, mutations related to the copper/zinc superoxide-dismutase-1 gene (SOD1), Tar DNA-binding protein gene (TARDBP) and DNA/RNA-binding protein FUS (fused in sarcoma) (3, 4). Only 5–10% of ALS cases are familiar, whereas the rest of ALS cases are sporadic, most of them with unknown cause.
The immune system has emerged as one of the key players linked to the development of neurodegeneration, such as in ALS (5–10). The immune system seems to have a dual role, polarizing its functional phenotype toward an inflammatory M1 phenotype or toward an anti-inflammatory M2 phenotype, depending on the particular neurodegenerative environment and by disease stage (7, 11). Alth...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
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