Modulation of neurodegeneration by molecular chaperones

PJ Muchowski, JL Wacker - Nature Reviews Neuroscience, 2005 - nature.com
Many neurodegenerative disorders are characterized by conformational changes in proteins
that result in misfolding, aggregation and intra-or extra-neuronal accumulation of amyloid …

Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease

M Katsuno, C Sang, H Adachi… - Proceedings of the …, 2005 - National Acad Sciences
Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused
by the expansion of a trinucleotide CAG repeat encoding the polyglutamine tract in the first …

[HTML][HTML] Geldanamycin induces heat shock protein 70 and protects against MPTP-induced dopaminergic neurotoxicity in mice

HY Shen, JC He, Y Wang, QY Huang… - Journal of Biological …, 2005 - ASBMB
As key molecular chaperone proteins, heat shock proteins (HSPs) represent an important
cellular protective mechanism against neuronal cell death in various models of neurological …

Regulation of heat shock gene transcription in neuronal cells

J Tonkiss, SK Calderwood - International journal of hyperthermia, 2005 - Taylor & Francis
The heat shock protein (HSP) molecular chaperones are the primary cellular defense
against damage to the proteome, initiating refolding of denatured proteins and regulating …

Extracellular heat shock protein 70: a critical component for motoneuron survival

MB Robinson, JL Tidwell, T Gould… - Journal of …, 2005 - Soc Neuroscience
The dependence of developing spinal motoneuron survival on a soluble factor (s) from their
target, muscle tissue is well established both in vivo and in vitro. Considering this apparent …

Intraneuronal β-amyloid expression downregulates the Akt survival pathway and blunts the stress response

J Magrané, KM Rosen, RC Smith, K Walsh… - Journal of …, 2005 - Soc Neuroscience
Early events in Alzheimer's disease (AD) pathogenesis implicate the accumulation of β-
amyloid (Aβ) peptide inside neurons in vulnerable brain regions. However, little is known …

[HTML][HTML] Aberrantly Increased Hydrophobicity Shared by Mutants of Cu, Zn-Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis*♦

A Tiwari, Z Xu, LJ Hayward - Journal of Biological Chemistry, 2005 - ASBMB
More than 100 different mutations in the gene encoding Cu, Zn-superoxide dismutase
(SOD1) cause preferential motor neuron degeneration in familial amyotrophic lateral …

Dopaminergic neurons in rat ventral midbrain cultures undergo selective apoptosis and form inclusions, but do not up‐regulate iHSP70, following proteasomal …

HJ Rideout, ICJ Lang‐Rollin, M Savalle… - Journal of …, 2005 - Wiley Online Library
Dysfunction of the ubiquitin‐dependent protein degradation system, either at the level of the
proteasome itself, or at the level of ubiquitination, may play a role in the pathogenesis of …

[HTML][HTML] Inhibition of chaperone activity is a shared property of several Cu, Zn-superoxide dismutase mutants that cause amyotrophic lateral sclerosis

H Tummala, C Jung, A Tiwari, CMJ Higgins… - Journal of Biological …, 2005 - ASBMB
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive motor neuron degeneration, paralysis, and death. Mutant Cu, Zn-superoxide …

Small heat shock proteins in inherited peripheral neuropathies

I Dierick, J Irobi, P De Jonghe, V Timmerman - Annals of medicine, 2005 - Taylor & Francis
Small heat shock proteins (small HSPs) are molecular chaperones that protect cells against
stress by assisting in the correct folding of denatured proteins and thus prevent aggregation …