(Q38820041)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

title

Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease (English)

1 reference

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18 March 2020

4

1 reference

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18 March 2020

Ilaria Tonazzini

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

Mauro Gemmi

8

1 reference

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18 March 2020

author name string

Valentina Cappello

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

Laura Marchetti

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

Paola Parlanti

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

Marco Cecchini

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

Vincenzo Piazza

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

language of work or name

English

0 references

publication date

5 December 2016

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

volume

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

page(s)

1

1 reference

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18 March 2020

issue

1

1 reference

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Creative Commons Attribution 4.0 International

18 March 2020

copyright license

start time

1 December 2016

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

exact match

https://scigraph.springernature.com/pub.10.1038/s41598-016-0001-8

0 references

cites work

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Pervasive supply of therapeutic lysosomal enzymes in the CNS of normal and Krabbe-affected non-human primates by intracerebral lentiviral gene therapy

9 September 2017

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Altered Trafficking and Processing of GALC Mutants Correlates with Globoid Cell Leukodystrophy Severity

9 September 2017

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Long-term Improvements in Lifespan and Pathology in CNS and PNS After BMT Plus One Intravenous Injection of AAVrh10-GALC in Twitcher Mice

9 September 2017

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9 September 2017

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Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy

9 September 2017

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Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy

9 September 2017

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Mechanism of neuromuscular dysfunction in Krabbe disease.

9 September 2017

1 reference

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Deficient nitric oxide signalling impairs skeletal muscle growth and performance: involvement of mitochondrial dysregulation

9 September 2017

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Role of extracellular calcium and mitochondrial oxygen species in psychosine-induced oligodendrocyte cell death

9 September 2017

1 reference

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History, genetic, and recent advances on Krabbe disease

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease

9 September 2017

1 reference

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Inhibition of angiogenesis by β-galactosylceramidase deficiency in globoid cell leukodystrophy.

9 September 2017

1 reference

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The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors

9 September 2017

1 reference

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The short-time structural plasticity of dendritic spines is altered in a model of Rett syndrome

9 September 2017

1 reference

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SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals

9 September 2017

1 reference

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The myelin mutants as models to study myelin repair in the leukodystrophies

9 September 2017

1 reference

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Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3.

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Motor neuron diversity in development and disease

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Psychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture.

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Negative regulation of myelination: relevance for development, injury, and demyelinating disease

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Biology and pathology of nonmyelinating Schwann cells

9 September 2017

1 reference

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Assembly, plasticity and selective vulnerability to disease of mouse neuromuscular junctions

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Molecular genetics of Krabbe disease (globoid cell leukodystrophy): diagnostic and clinical implications

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease

9 September 2017

1 reference

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The twitcher mouse. A model of human globoid cell leukodystrophy (krabbe's disease)

9 September 2017

1 reference

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The twitcher mouse. An alteration of the unmyelinated fibers in the PNS

9 September 2017

1 reference

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Simplified mammalian DNA isolation procedure

9 September 2017

1 reference

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Psychosine induces the dephosphorylation of neurofilaments by deregulation of PP1 and PP2A phosphatases

9 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Region- and age-dependent alterations of glial-neuronal metabolic interactions correlate with CNS pathology in a mouse model of globoid cell leukodystrophy.

9 September 2017

1 reference

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Autophagy as a new therapeutic target in Duchenne muscular dystrophy

17 June 2018

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Oxidative stress as a therapeutic target in globoid cell leukodystrophy.

17 June 2018

1 reference

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The twitcher mouse: accumulation of galactosylsphingosine and pathology of the sciatic nerve.

17 June 2018

1 reference

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Pharmacological chaperones increase residual β-galactocerebrosidase activity in fibroblasts from Krabbe patients.

17 June 2018

1 reference

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Axonopathy is a compounding factor in the pathogenesis of Krabbe disease.

22 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5431369

Sustained axon-glial signaling induces Schwann cell hyperproliferation, Remak bundle myelination, and tumorigenesis.

22 August 2018

1 reference

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An intrinsic distinction in neuromuscular junction assembly and maintenance in different skeletal muscles

22 August 2018

1 reference

12 December 2020

A role for Schwann cell-derived neuregulin-1 in remyelination

1 reference

12 December 2020

RP-CARS reveals molecular spatial order anomalies in myelin of an animal model of Krabbe disease

1 reference

12 December 2020

Hereditary leucodystrophy in the mouse: the new mutant twitcher

1 reference

12 December 2020

Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

1 reference

12 December 2020

Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model of ALS

1 reference

12 December 2020

Molecular genetics of Krabbe disease (globoid cell leukodystrophy): Diagnostic and clinical implications

1 reference

Crossref

https://api.crossref.org/works/10.1038%2FS41598-016-0001-8

7 January 2021

inferred from DOI database lookup

Identifiers

DOI

10.1038/S41598-016-0001-8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

18 March 2020

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:28442746%20AND%20SRC:MED&resulttype=core&format=json

18 March 2020

1 reference