Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

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

Neurobiology of disease - 1 Aug 2012

Srivastava Amit K, Renusch Samantha R, Naiman Nicole E, Gu Shuping, Sneh Amita, Arnold W David, Sahenk Zarife, Kolb Stephen J

Abstract excerpt

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...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Axons
Share to Charcot-Marie-Tooth Disease

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Heat-Shock Proteins

Share to HSP27 Heat-Shock Proteins

Share to Molecular Chaperones

Share to Motor Neuron Disease

Share to Motor Neurons

Share to Random Allocation

Source

Open in PubMed Open full text

Identifiers

PMID: 22521462
DOI: 10.1016/j.nbd.2012.03.035
PMCID: PMC3367118

Genes

HSPB1

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice.PMID 22521462Read ↗

Mutant HSPB8 causes motor neuron-specific neurite degeneration.Semantic proximity Read ↗

A Charcot-Marie-Tooth-Causing Mutation in HSPB1 Decreases Cell Adaptation to Repeated Stress by Disrupting Autophagic Clearance of Misfolded Proteins.Semantic proximity Read ↗

Characterization of New Transgenic Mouse Models for Two Charcot-Marie-Tooth-Causing HspB1 Mutations using the Rosa26 Locus.Semantic proximity Read ↗

A mutation in the small heat-shock protein HSPB1 leading to distal hereditary motor neuronopathy disrupts neurofilament assembly and the axonal transport of specific cellular cargoes.Semantic proximity Read ↗

Mutations in heat shock protein beta-1 (HSPB1) are associated with a range of clinical phenotypes related to different patterns of motor neuron dysfunction: A case series.Semantic proximity Read ↗

Overexpression of mutant HSP27 causes axonal neuropathy in mice.Semantic proximity Read ↗

Stress-Driven Selective Neuronal Vulnerability in Charcot-Marie-Tooth Disease: From Prodromal Pathology to Therapeutic Implications.Semantic proximity Read ↗

Mutations in HspB1 and hereditary neuropathies.Semantic proximity Read ↗

HSPB1 mutations causing hereditary neuropathy in humans disrupt non-cell autonomous protection of motor neurons.Semantic proximity Read ↗

Neuronal diseases: small heat shock proteins calm your nerves.Semantic proximity Read ↗

Small heat-shock protein HSPB1 mutants stabilize microtubules in Charcot-Marie-Tooth neuropathy.Semantic proximity Read ↗

Small heat shock proteins in neurodegenerative diseases.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.