Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice

Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice

The small heat shock protein HSPB1 is a multifunctional, α-crystallin-based protein that has been shown to be neuroprotective in animal models of motor neuron disease and peripheral nerve injury. Missense mutations in HSPB1 result in axonal Charcot–Marie–Tooth disease with minimal sensory involvemen...

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Bibliographic Details
Main Authors: Amit K. Srivastava, Samantha R. Renusch, Nicole E. Naiman, Shuping Gu, Amita Sneh, W. David Arnold, Zarife Sahenk, Stephen J. Kolb
Format: Article
Language:English
Published: Elsevier 2012-08-01
Series:Neurobiology of Disease
Subjects:
Hereditary motor neuropathy
Charcot–Marie–Tooth disease
Small heat shock protein
Prion protein promoter
Axonal degeneration
Motor neuron disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996112001246

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http://www.sciencedirect.com/science/article/pii/S0969996112001246

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