ArticleNeurobiology of disease2012
Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice.
Article in Neurobiology of disease, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 37 citations in OpenAlex.
- Review
- Article
- AAV1.NT-3 gene therapy for X-linked Charcot-Marie-Tooth neuropathy type 1.Gene therapy · 2022Article
- Apolipoprotein E4 Effects a Distinct Transcriptomic Profile and Dendritic Arbor Characteristics in Hippocampal Neurons CulturedFrontiers in aging neuroscience · 2022Article
- Insights on Human Small Heat Shock Proteins and Their Alterations in Diseases.Frontiers in molecular biosciences · 2022Review
- Animal Models as a Tool to Design Therapeutical Strategies for CMT-like Hereditary Neuropathies.Brain sciences · 2021Review
- Mutations in HspB1 and hereditary neuropathies.Cell stress & chaperones · 2020Review
- Decoding the relationship between ageing and amyotrophic lateral sclerosis: a cellular perspective.Brain : a journal of neurology · 2020Review
- Neuromuscular Diseases Due to Chaperone Mutations: A Review and Some New Results.International journal of molecular sciences · 2020Review
- Novel HDAC6 Inhibitors Increase Tubulin Acetylation and Rescue Axonal Transport of Mitochondria in a Model of Charcot-Marie-Tooth Type 2F.ACS chemical neuroscience · 2020Article
- Controlling properties of human neural progenitor cells using 2D and 3D conductive polymer scaffolds.Scientific reports · 2019Article
- Muscle contractility dysfunction precedes loss of motor unit connectivity in SOD1(G93A) mice.Muscle & nerve · 2019Article
- In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration.Journal of visualized experiments : JoVE · 2018Article
- Decreased ceramide underlies mitochondrial dysfunction in Charcot-Marie-Tooth 2F.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2018Article
- HSPB1 mutations causing hereditary neuropathy in humans disrupt non-cell autonomous protection of motor neurons.Experimental neurology · 2017Article
- A novel p.T139M mutation in HSPB1 highlighting the phenotypic spectrum in a family.Brain and behavior · 2017Article
- Small heat shock proteins in ageing and age-related diseases.Cell stress & chaperones · 2017Review
- Pilot phenotype and natural history study of hereditary neuropathies caused by mutations in the HSPB1 gene.Neuromuscular disorders : NMD · 2017Article
- Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles.Journal of visualized experiments : JoVE · 2015Article
- Overexpression of mutant HSP27 causes axonal neuropathy in mice.Journal of biomedical science · 2015Article
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
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 involvement (CMT2F) and distal hereditary motor neuropathy type 2 (dHMN-II). These disorders are characterized by a selective loss of motor axons in peripheral nerve resulting in distal muscle weakness and often severe disability. To investigate the pathogenic mechanisms of HSPB1 mutations in motor neurons in vivo, we have developed and characterized transgenic PrP-HSPB1 and PrP-HSPB1(R136W) mice. These mice express the human HSPB1 protein throughout the nervous system including in axons of peripheral nerve. Although both mouse strains lacked obvious motor deficits, the PrP-HSPB1(R136W) mice developed an age-dependent motor axonopathy. Mutant mice showed axonal pathology in spinal cord and peripheral nerve with evidence of impaired neurofilament cytoskeleton, associated with organelle accumulation. Accompanying these findings, increases in the number of Schmidt-Lanterman incisures, as evidence of impaired axon-Schwann cell interactions, were present. These observations suggest that overexpression of HSPB1(R136W) in neurons is sufficient to cause pathological and electrophysiological changes in mice that are seen in patients with hereditary motor neuropathy.
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Registered trials
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