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Article

Plastin 3 Promotes Motor Neuron Axonal Growth and Extends Survival in a Mouse Model of Spinal Muscular Atrophy

31 Jan 2018

Abstract excerpt

), leading to reduced levels of SMN protein in the CNS. The actin-binding protein plastin 3 (PLS3) has been reported as a modifier for SMA, making it a potential therapeutic target. Here, we show reduced levels of PLS3 protein in the brain and spinal cord of a mouse model of SMA. Our study also revealed that lentiviral-mediated PLS3 expression restored axonal length in cultured Smn-deficient motor neurons....

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Identifiers

PMID: 29552580
DOI: 10.1016/j.omtm.2018.01.007

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Related research

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Plastin 3 Promotes Motor Neuron Axonal Growth and Extends Survival in a Mouse Model of Spinal Muscular AtrophyPMID 29552580Read ↗

Plastin-3 extends survival and reduces severity in mouse models of spinal muscular atrophySemantic proximity Read ↗

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Plastin 3 ameliorates spinal muscular atrophy via delayed axon pruning and improves neuromuscular junction functionality.Semantic proximity Read ↗

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CHP1 reduction ameliorates spinal muscular atrophy pathology by restoring calcineurin activity and endocytosisSemantic proximity Read ↗

Genetic modifiers ameliorate endocytic and neuromuscular defects in a model of spinal muscular atrophySemantic proximity Read ↗

The Power of Human Protective Modifiers: PLS3 and CORO1C Unravel Impaired Endocytosis in Spinal Muscular Atrophy and Rescue SMA Phenotype.Semantic proximity Read ↗

Calcium binding is essential for plastin 3 function in Smn-deficient motoneurons.Semantic proximity Read ↗

PLS3 expression and SMA phenotype: a commentary on correlation of PLS3 expression with disease severity in children with spinal muscular atrophy.Semantic proximity Read ↗

Plastin 3 is upregulated in iPSC-derived motoneurons from asymptomatic SMN1-deleted individuals.Semantic proximity Read ↗

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