Is Innate Immunity And Inflammasomes Involved In Pathogenesis of Amyotrophic Lateral Sclerosis (ALS)?

Is Innate Immunity And Inflammasomes Involved In Pathogenesis of Amyotrophic Lateral Sclerosis (ALS)?
Recent Pat Endocr Metab Immune Drug Discov. 2015 Apr 7;
Authors: Volpe CM, Nogueira-Machado JA
Abstract
Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig's disease is an axonopathy with adult-onset, progressive and irreversible degeneration of upper and lower motor neurons. Around 90% of ALS is considered as sporadic ALS (sALS) without apparent genetic cause while in the familial type of ALS (fALS) at least one affected blood relative needs to be identified. Both sALS and fALS show similar progression and pathological profile. Biochemical and immunological roles have been reported for both types of ALS. It has been suggested that mutation in SOD1 gene would be responsible for the oxidative stress and neurotoxicity. Besides, oxidative stress, protein aggregation, altered cholinergic synapse, neuro-inflammation and production of pro-inflammatory cytokines have also been reported. Thus, the focus of the present review was on biochemical and immunological biomarkers and pathogenic mechanism. Regulatory T cells, pro-inflammatory cytokines and activation of pro-inflammatory signaling pathway are discussed. The activation of NRL inflammasomes in ALS and the involvement of IL-18, IL-1β and capases-1are also suggested. The presence and importance of HMGB-1 (DAMP) and activation of Toll-like receptors and/or RAGE also are envisaged. The paten...

Source: Recent Patents on Endocrine, Metabolic and Immune Drug Discovery - June 4, 2015 Category: Endocrinology Tags: Recent Pat Endocr Metab Immune Drug Discov Source Type: research