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Article

A neurotoxic peripherin splice variant in a mouse model of ALS.

The Journal of cell biology - 17 Mar 2003

Robertson Janice, Doroudchi Mohammad M, Nguyen Minh Dang, Durham Heather D, Strong Michael J, Shaw Gerry, Julien Jean-Pierre, Mushynski Walter E

Abstract excerpt

Peripherin, a neuronal intermediate filament (nIF) protein found associated with pathological aggregates in motor neurons of patients with amyotrophic lateral sclerosis (ALS) and of transgenic mice overexpressing mutant superoxide dismutase-1 (SOD1G37R), induces the selective degeneration of motor neurons when overexpressed in transgenic mice. Mouse peripherin is unique compared with other nIF proteins in that...

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Identifiers

PMID: 12642616
DOI: 10.1083/jcb.200205027
PMCID: PMC2173778

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.

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A neurotoxic peripherin splice variant in a mouse model of ALS.PMID 12642616Read ↗

An Aggregate-Inducing Peripherin Isoform Generated through Intron Retention Is Upregulated in Amyotrophic Lateral Sclerosis and Associated with Disease PathologySemantic proximity Read ↗

A two-hybrid screen identifies an unconventional role for the intermediate filament peripherin in regulating the subcellular distribution of the SNAP25-interacting protein, SIP30.Semantic proximity Read ↗

Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type PeripherinSemantic proximity Read ↗

A Frameshift Deletion in Peripherin Gene Associated with Amyotrophic Lateral SclerosisSemantic proximity Read ↗

ALS mouse model SOD1G93Adisplays early pathology of sensory small fibers associated to accumulation of a neurotoxic splice variant of peripherinSemantic proximity Read ↗

A pathogenic peripherin gene mutation in a patient with amyotrophic lateral sclerosis.Semantic proximity Read ↗

Defective axonal transport of neurofilament proteins in neurons overexpressing peripherinSemantic proximity Read ↗

Expression of peripherin in ubiquinated inclusions of amyotrophic lateral sclerosisSemantic proximity Read ↗

Formation of Intermediate Filament Protein Aggregates with Disparate Effects in Two Transgenic Mouse Models Lacking the Neurofilament Light SubunitSemantic proximity Read ↗

Ultrastructural studies of carboxyl-terminal truncation mutants of the neuronal intermediate filament protein peripherin.Semantic proximity Read ↗

Modulating Expression of Peripherin/<i>rds</i>in Transgenic Mice: Critical Levels and the Effect of OverexpressionSemantic proximity Read ↗

Peripherin, a new promising biomarker in neurological disordersSemantic proximity Read ↗

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