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Article

Microtubule-targeting drugs rescue axonal swellings in cortical neurons from spastin knockout mice.

Disease models & mechanisms - 1 Jan 2013

Fassier Coralie, Tarrade Anne, Peris Leticia, Courageot Sabrina, Mailly Philippe, Dalard Cécile, Delga Stéphanie, Roblot Natacha, Lefèvre Julien, Job Didier, Hazan Jamilé, Curmi Patrick A, Melki Judith

Abstract excerpt

Mutations in SPG4, encoding the microtubule-severing protein spastin, are responsible for the most frequent form of hereditary spastic paraplegia (HSP), a heterogeneous group of genetic diseases characterized by degeneration of the corticospinal tracts. We previously reported that mice harboring a deletion in Spg4, generating a premature stop codon, develop progressive axonal degeneration characterized by focal...

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Axonal Transport
Axons
Models, Neurological
Nocodazole
Paclitaxel

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Spastic Paraplegia, Hereditary

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Identifiers

PMID: 22773755
DOI: 10.1242/dmm.008946
PMCID: PMC3529340

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Microtubule-targeting drugs rescue axonal swellings in cortical neurons from spastin knockout mice.PMID 22773755Read ↗

Loss of spastin function results in disease-specific axonal defects in human pluripotent stem cell-based models of hereditary spastic paraplegia.Semantic proximity Read ↗

A mutation of spastin is responsible for swellings and impairment of transport in a region of axon characterized by changes in microtubule composition.Semantic proximity Read ↗

Pathogenic mutation of spastin has gain-of-function effects on microtubule dynamics.Semantic proximity Read ↗

Genetic and chemical modulation of spastin-dependent axon outgrowth in zebrafish embryos indicates a role for impaired microtubule dynamics in hereditary spastic paraplegia.Semantic proximity Read ↗

Spastin Is Required to Prevent SPAST-Related Demyelination.Semantic proximity Read ↗

Linking axonal degeneration to microtubule remodeling by Spastin-mediated microtubule severing.Semantic proximity Read ↗

Gene dosage-dependent rescue of HSP neurite defects in SPG4 patients' neuronsSemantic proximity Read ↗

Genotype–Phenotype Distinctions in Spastic Paraplegia 4 Reveal HDAC6 as a Therapeutic TargetSemantic proximity Read ↗

Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients.Semantic proximity Read ↗

Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics.Semantic proximity Read ↗

Mutant spastin proteins promote deficits in axonal transport through an isoform-specific mechanism involving casein kinase 2 activation.Semantic proximity Read ↗

Drosophila spastin regulates synaptic microtubule networks and is required for normal motor function.Semantic proximity Read ↗

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