(Q37625840)

English

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

scientific article published on February 2014

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Europe PubMed Central

PMC publication ID

21 August 2017

Loss of spastin function results in disease-specific axonal defects in human pluripotent stem cell-based models of hereditary spastic paraplegia (English)

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

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author name string

Kyle R Denton

1

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Europe PubMed Central

PMC publication ID

21 August 2017

Ling Lei

2

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Europe PubMed Central

PMC publication ID

21 August 2017

Jeremy Grenier

3

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

Vladimir Rodionov

4

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

Craig Blackstone

5

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Europe PubMed Central

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21 August 2017

Xue-Jun Li

6

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Europe PubMed Central

PMC publication ID

21 August 2017

publication date

1 February 2014

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Europe PubMed Central

PMC publication ID

21 August 2017

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Europe PubMed Central

PMC publication ID

21 August 2017

32

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Europe PubMed Central

PMC publication ID

21 August 2017

2

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Europe PubMed Central

PMC publication ID

21 August 2017

414-423

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Europe PubMed Central

PMC publication ID

21 August 2017

describes a project that uses

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Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3947148/fullTextXML

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A patient-derived stem cell model of hereditary spastic paraplegia with SPAST mutations

1 reference

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

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Microtubule-severing enzymes at the cutting edge

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Mutations in the ER-shaping protein reticulon 2 cause the axon-degenerative disorder hereditary spastic paraplegia type 12

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Hereditary spastic paraplegia proteins REEP1, spastin, and atlastin-1 coordinate microtubule interactions with the tubular ER network

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Axonal transport defects in neurodegenerative diseases

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

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Critical roles for microtubules in axonal development and disease

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Parkinson's disease patient-derived induced pluripotent stem cells free of viral reprogramming factors

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Spastin couples microtubule severing to membrane traffic in completion of cytokinesis and secretion

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Structural basis for midbody targeting of spastin by the ESCRT-III protein CHMP1B

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Disease-specific induced pluripotent stem cells

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Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons

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Quantitative and functional analyses of spastin in the nervous system: implications for hereditary spastic paraplegia

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The microtubule-severing proteins spastin and katanin participate differently in the formation of axonal branches

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Induction of pluripotent stem cells from adult human fibroblasts by defined factors

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Induced pluripotent stem cell lines derived from human somatic cells

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Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

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Human spastin has multiple microtubule-related functions

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Hereditary spastic paraplegia

1 reference

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Spastin, the protein mutated in autosomal dominant hereditary spastic paraplegia, is involved in microtubule dynamics

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Spastin, a new AAA protein, is altered in the most frequent form of autosomal dominant spastic paraplegia

1 reference

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Individual microtubules in the axon consist of domains that differ in both composition and stability

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Cell biology: Tubulin acetylation and cell motility

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1 September 2018

Identifiers

10.1002/STEM.1569

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

21 August 2017

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