Molecular chaperones as therapeutic targets in amyotrophic lateral sclerosis.
Neurodegenerative diseases are characterized by a number of common hallmarks, such as the presence of intracellular aggregates and activation of the apoptotic cell-death pathway. Intracellular chaperones, responsible for protein integrity and structural repair, may play a crucial role in the progression of a disease. In this paper, we aim to summarize our understanding of the role and potential of a particular family of chaperones, the heat-shock proteins, in neurodegeneration, by focusing our discussion on models of motoneuron death.
Kalmar
B
B
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK.
Kieran
D
D
Greensmith
L
L
D018450:Disease Progression; D006360:Heat-Shock Proteins / Q000502:physiology*; D006801:Humans; D018832:Molecular Chaperones / Q000502:physiology*; D016472:Motor Neuron Disease / Q000628:therapy*; D009046:Motor Neurons / Q000473:pathology*; D010523:Peripheral Nervous System Diseases / Q000473:pathology
D006360:Heat-Shock Proteins; D018832:Molecular Chaperones