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

20 Feb 2008

Abstract excerpt

The neuronal intermediate filament protein peripherin is a component of ubiquitinated inclusions and of axonal spheroids in amyotrophic lateral sclerosis (ALS). Overexpression of peripherin causes motor neuron degeneration in transgenic mice and variations within the peripherin gene have been identified in ALS cases. We have shown previously the abnormal expression of a neurotoxic peripherin splice variant in...

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PMID: 18287500
DOI: 10.1523/jneurosci.3222-07.2008

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An Aggregate-Inducing Peripherin Isoform Generated through Intron Retention Is Upregulated in Amyotrophic Lateral Sclerosis and Associated with Disease PathologyPMID 18287500Read ↗

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

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

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