Heat Shock Proteins: Old and Novel Roles in Neurodegenerative Diseases in the Central Nervous System

Title:Heat Shock Proteins: Old and Novel Roles in Neurodegenerative Diseases in the Central Nervous System

Volume: 16 Issue: 3

Author(s): Johannes M. van Noort, Marianna Bugiani and Sandra Amor*

Affiliation:

• Department of Pathology, VU University Medical Centre De Boelelaan 1117, 1081 HV Amsterdam,Netherlands

Keywords: AD, ALS, chaperokines, chaperones, heat shock proteins, immune regulation, MS, neurodegeneration.

Abstract: Heat shock proteins (HSPs) are families of molecular chaperones that play important homeostatic functions in the central nervous system (CNS) by preventing protein misfolding, promoting degradation of improperly folded proteins, and protecting against apoptosis and inflammatory damage especially during hyperthermia, hypoxia, or oxidative stress. Under stress conditions, HSPs are upregulated to protect cells from damage that accumulates during ageing as well as pathological conditions. An important, yet frequently overlooked function of some HSPs is their ability to function as extracellular messengers (also termed chaperokines) that modulate immune responses within the CNS. Given the strong association between protein aggregation, innate immune cell activation and neurodegeneration, the expression and roles of HSPs in the CNS is attracting attention in many neurodegenerative disorders including inflammatory diseases such as multiple sclerosis, protein folding diseases such as Alzheimer’s disease and amyotrophic lateral sclerosis, and genetic white matter diseases. This is especially so since several studies show that HSPs act therapeutically by modulating innate immune activation and may thus serve as neuroprotective agents.

Cite this article as:

van Noort M. Johannes, Bugiani Marianna and Amor Sandra*, Heat Shock Proteins: Old and Novel Roles in Neurodegenerative Diseases in the Central Nervous System, CNS & Neurological Disorders - Drug Targets 2017; 16 (3) . https://dx.doi.org/10.2174/1871527315666161031125317