PropertyValue
?:abstract
  • Sphingolipidoses are diseases caused by mutations in genes responsible for sphingolipid degradation and thereby lead to sphingolipid accumulation. Most sphingolipidoses have a neurodegenerative manifestation characterized by innate immune activation in the brain. However, the role of the immune response in disease progression is ill-understood. In contrast to infectious diseases, immune activation is unable to eliminate the offending agent in sphingolipidoses resulting in ineffective, chronic inflammation. This paradox begs two fundamental questions: why has this immune response evolved in sphingolipidoses? What role does it play in disease progression? Here, starting from the observation that sphingolipids are elevated also in infectious diseases, I discuss the possibility that activation of the brain immune response by sphingolipids has evolved as a part of the immune response against pathogens and plays no major role in sphingolipidoses.
is ?:annotates of
?:creator
?:doi
?:doi
  • 10.1002/1873-3468.13980
?:journal
  • FEBS_letters
?:license
  • unk
?:pmid
?:pmid
  • 33131047.0
?:publication_isRelatedTo_Disease
is ?:relation_isRelatedTo_publication of
?:source
  • Medline
?:title
  • The role of brain innate immune response in lysosomal storage disorders: fundamental process or evolutionary side-effect?
?:type
?:year
  • 2020-10-31

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