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The role of innate immunity in mucopolysaccharide diseases
Author(s) -
Parker Helen,
Bigger Brian W.
Publication year - 2019
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/jnc.14632
Subject(s) - innate immune system , inflammation , immune system , immunology , mucopolysaccharidosis , immunity , biology , glycosaminoglycan , disease , neuroscience , medicine , pathology , biochemistry
Mucopolysaccharidoses are lysosomal storage disorders characterised by accumulation of abnormal pathological glycosaminoglycans, cellular dysfunction and widespread inflammation, resulting in progressive cognitive and motor decline. Lysosomes are important mediators of immune cell function, and therefore accumulation of glycosaminoglycans ( GAG s) and other abnormal substrates could affect immune function and directly impact on disease pathogenesis. This review summarises current knowledge with regard to inflammation in mucopolysaccharidosis, with an emphasis on the brain and outlines a potential role for GAG s in induction of inflammation. We propose a model by which the accumulation of GAG s and other factors may impact on innate immune signalling with particular focus on the Toll‐like receptor 4 pathway. Innate immunity appears to have a dominating role in mucopolysaccharidosis; however, furthering understanding of innate immune signalling would have significant impact on highlighting novel anti‐inflammatory therapeutics for use in mucopolysaccharide diseases.This article is part of the Special Issue “Lysosomal Storage Disorders” .