Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2016-12

AUTHORS

Valentina Cappello, Laura Marchetti, Paola Parlanti, Silvia Landi, Ilaria Tonazzini, Marco Cecchini, Vincenzo Piazza, Mauro Gemmi

ABSTRACT

Krabbe disease (KD) is a neurodegenerative disorder caused by the lack of β- galactosylceramidase enzymatic activity and by widespread accumulation of the cytotoxic galactosyl-sphingosine in neuronal, myelinating and endothelial cells. Despite the wide use of Twitcher mice as experimental model for KD, the ultrastructure of this model is partial and mainly addressing peripheral nerves. More details are requested to elucidate the basis of the motor defects, which are the first to appear during KD onset. Here we use transmission electron microscopy (TEM) to focus on the alterations produced by KD in the lower motor system at postnatal day 15 (P15), a nearly asymptomatic stage, and in the juvenile P30 mouse. We find mild effects on motorneuron soma, severe ones on sciatic nerves and very severe effects on nerve terminals and neuromuscular junctions at P30, with peripheral damage being already detectable at P15. Finally, we find that the gastrocnemius muscle undergoes atrophy and structural changes that are independent of denervation at P15. Our data further characterize the ultrastructural analysis of the KD mouse model, and support recent theories of a dying-back mechanism for neuronal degeneration, which is independent of demyelination. More... »

PAGES

1

References to SciGraph publications

Identifiers

URI

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

DOI

http://dx.doi.org/10.1038/s41598-016-0001-8

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1056467545


Indexing Status Check whether this publication has been indexed by Scopus and Web Of Science using the SN Indexing Status Tool
Incoming Citations Browse incoming citations for this publication using opencitations.net

JSON-LD is the canonical representation for SciGraph data.

TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT

[
  {
    "@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json", 
    "about": [
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1109", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Neurosciences", 
        "type": "DefinedTerm"
      }, 
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Medical and Health Sciences", 
        "type": "DefinedTerm"
      }
    ], 
    "author": [
      {
        "affiliation": {
          "name": [
            "Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Cappello", 
        "givenName": "Valentina", 
        "id": "sg:person.0704765127.66", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0704765127.66"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "name": [
            "Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Marchetti", 
        "givenName": "Laura", 
        "id": "sg:person.0676657141.49", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0676657141.49"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Scuola Normale Superiore di Pisa", 
          "id": "https://www.grid.ac/institutes/grid.6093.c", 
          "name": [
            "Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy", 
            "NEST, Scuola Normale Superiore, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Parlanti", 
        "givenName": "Paola", 
        "id": "sg:person.01352275273.03", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01352275273.03"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "name": [
            "NEST, Istituto Nanoscienze-CNR, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Landi", 
        "givenName": "Silvia", 
        "id": "sg:person.01100741224.17", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01100741224.17"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Fondazione Umberto Veronesi", 
          "id": "https://www.grid.ac/institutes/grid.478935.4", 
          "name": [
            "NEST, Istituto Nanoscienze-CNR, Pisa, Italy", 
            "Fondazione Umberto Veronesi, Milano, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Tonazzini", 
        "givenName": "Ilaria", 
        "id": "sg:person.0601671705.59", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0601671705.59"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Scuola Normale Superiore di Pisa", 
          "id": "https://www.grid.ac/institutes/grid.6093.c", 
          "name": [
            "NEST, Scuola Normale Superiore, Pisa, Italy", 
            "NEST, Istituto Nanoscienze-CNR, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Cecchini", 
        "givenName": "Marco", 
        "id": "sg:person.0632662545.29", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0632662545.29"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "name": [
            "Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Piazza", 
        "givenName": "Vincenzo", 
        "id": "sg:person.01331013067.25", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01331013067.25"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "name": [
            "Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Gemmi", 
        "givenName": "Mauro", 
        "id": "sg:person.0741334155.71", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0741334155.71"
        ], 
        "type": "Person"
      }
    ], 
    "citation": [
      {
        "id": "https://doi.org/10.1016/0006-8993(88)90835-9", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1000076080"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.nbd.2012.01.013", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1005580237"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/srep00045", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1005632431", 
          "https://doi.org/10.1038/srep00045"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/cddis.2014.483", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1005725448", 
          "https://doi.org/10.1038/cddis.2014.483"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.nbd.2015.08.004", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1005813908"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1046/j.1471-4159.1996.66031118.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1007273365"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/jbio.201500305", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1009600906"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nn1653", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1010416853", 
          "https://doi.org/10.1038/nn1653"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nn1653", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1010416853", 
          "https://doi.org/10.1038/nn1653"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.15252/emmm.201505850", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1010545031"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s13311-011-0080-y", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1010548540", 
          "https://doi.org/10.1007/s13311-011-0080-y"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/(sici)1098-1004(1997)10:4<268::aid-humu2>3.0.co;2-d", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1013204335"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.expneurol.2012.07.013", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1013420690"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1023/b:neur.0000020628.36013.88", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1014276565", 
          "https://doi.org/10.1023/b:neur.0000020628.36013.88"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.5597-08.2009", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1015262280"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/brain/awt215", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016427155"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.gene.2014.09.046", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016509648"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.1753-09.2009", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1017032597"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/glia.20761", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1017134781"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.nbd.2014.02.012", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1017740527"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1042/an20110019", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1018677978"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1146/annurev.neuro.051508.135722", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1020995537"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1038/mt.2015.145", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1029771913"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/s0896-6273(02)00670-0", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1030361991"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.4199-14.2015", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1033568851"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/hmg/ddv086", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1033869737"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/nar/19.15.4293", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1034171019"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1186/s13395-014-0022-6", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1034682712", 
          "https://doi.org/10.1186/s13395-014-0022-6"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1186/s13395-014-0022-6", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1034682712", 
          "https://doi.org/10.1186/s13395-014-0022-6"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.3095-15.2016", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1038619602"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nn.3281", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1042231649", 
          "https://doi.org/10.1038/nn.3281"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.0217-13.2013", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1043478470"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1371/journal.pone.0026164", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1043971475"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.ymgme.2014.05.009", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1045807891"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/glia.20778", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1046916402"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1038/jcbfm.2013.64", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1048484759"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1038/jcbfm.2013.64", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1048484759"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s00401-011-0814-2", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1049873905", 
          "https://doi.org/10.1007/s00401-011-0814-2"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1523/jneurosci.2431-14.2015", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1051295323"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.mcn.2012.07.003", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1051714196"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1002/jnr.23744", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1052425771"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/brain/103.3.695", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1059437410"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/brain/39.1-2.74", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1059440791"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1079702738", 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1081997437", 
        "type": "CreativeWork"
      }
    ], 
    "datePublished": "2016-12", 
    "datePublishedReg": "2016-12-01", 
    "description": "Krabbe disease (KD) is a neurodegenerative disorder caused by the lack of \u03b2- galactosylceramidase enzymatic activity and by widespread accumulation of the cytotoxic galactosyl-sphingosine in neuronal, myelinating and endothelial cells. Despite the wide use of Twitcher mice as experimental model for KD, the ultrastructure of this model is partial and mainly addressing peripheral nerves. More details are requested to elucidate the basis of the motor defects, which are the first to appear during KD onset. Here we use transmission electron microscopy (TEM) to focus on the alterations produced by KD in the lower motor system at postnatal day 15 (P15), a nearly asymptomatic stage, and in the juvenile P30 mouse. We find mild effects on motorneuron soma, severe ones on sciatic nerves and very severe effects on nerve terminals and neuromuscular junctions at P30, with peripheral damage being already detectable at P15. Finally, we find that the gastrocnemius muscle undergoes atrophy and structural changes that are independent of denervation at P15. Our data further characterize the ultrastructural analysis of the KD mouse model, and support recent theories of a dying-back mechanism for neuronal degeneration, which is independent of demyelination.", 
    "genre": "research_article", 
    "id": "sg:pub.10.1038/s41598-016-0001-8", 
    "inLanguage": [
      "en"
    ], 
    "isAccessibleForFree": true, 
    "isPartOf": [
      {
        "id": "sg:journal.1045337", 
        "issn": [
          "2045-2322"
        ], 
        "name": "Scientific Reports", 
        "type": "Periodical"
      }, 
      {
        "issueNumber": "1", 
        "type": "PublicationIssue"
      }, 
      {
        "type": "PublicationVolume", 
        "volumeNumber": "6"
      }
    ], 
    "name": "Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease", 
    "pagination": "1", 
    "productId": [
      {
        "name": "readcube_id", 
        "type": "PropertyValue", 
        "value": [
          "ca46b7f85d1ce1378225471d9d461dccd0e1bece53b300b9ac0b890809838187"
        ]
      }, 
      {
        "name": "doi", 
        "type": "PropertyValue", 
        "value": [
          "10.1038/s41598-016-0001-8"
        ]
      }, 
      {
        "name": "dimensions_id", 
        "type": "PropertyValue", 
        "value": [
          "pub.1056467545"
        ]
      }
    ], 
    "sameAs": [
      "https://doi.org/10.1038/s41598-016-0001-8", 
      "https://app.dimensions.ai/details/publication/pub.1056467545"
    ], 
    "sdDataset": "articles", 
    "sdDatePublished": "2019-04-10T20:35", 
    "sdLicense": "https://scigraph.springernature.com/explorer/license/", 
    "sdPublisher": {
      "name": "Springer Nature - SN SciGraph project", 
      "type": "Organization"
    }, 
    "sdSource": "s3://com-uberresearch-data-dimensions-target-20181106-alternative/cleanup/v134/2549eaecd7973599484d7c17b260dba0a4ecb94b/merge/v9/a6c9fde33151104705d4d7ff012ea9563521a3ce/jats-lookup/v90/0000000001_0000000264/records_8684_00000426.jsonl", 
    "type": "ScholarlyArticle", 
    "url": "http://www.nature.com/articles/s41598-016-0001-8"
  }
]
 

Download the RDF metadata as:  json-ld nt turtle xml License info

HOW TO GET THIS DATA PROGRAMMATICALLY:

JSON-LD is a popular format for linked data which is fully compatible with JSON.

curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1038/s41598-016-0001-8'

N-Triples is a line-based linked data format ideal for batch operations.

curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1038/s41598-016-0001-8'

Turtle is a human-readable linked data format.

curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1038/s41598-016-0001-8'

RDF/XML is a standard XML format for linked data.

curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1038/s41598-016-0001-8'


 

This table displays all metadata directly associated to this object as RDF triples.

257 TRIPLES      21 PREDICATES      69 URIs      19 LITERALS      7 BLANK NODES

Subject Predicate Object
1 sg:pub.10.1038/s41598-016-0001-8 schema:about anzsrc-for:11
2 anzsrc-for:1109
3 schema:author Nd2f02c8078384814bbec4ca9554f3ab1
4 schema:citation sg:pub.10.1007/s00401-011-0814-2
5 sg:pub.10.1007/s13311-011-0080-y
6 sg:pub.10.1023/b:neur.0000020628.36013.88
7 sg:pub.10.1038/cddis.2014.483
8 sg:pub.10.1038/nn.3281
9 sg:pub.10.1038/nn1653
10 sg:pub.10.1038/srep00045
11 sg:pub.10.1186/s13395-014-0022-6
12 https://app.dimensions.ai/details/publication/pub.1079702738
13 https://app.dimensions.ai/details/publication/pub.1081997437
14 https://doi.org/10.1002/(sici)1098-1004(1997)10:4<268::aid-humu2>3.0.co;2-d
15 https://doi.org/10.1002/glia.20761
16 https://doi.org/10.1002/glia.20778
17 https://doi.org/10.1002/jbio.201500305
18 https://doi.org/10.1002/jnr.23744
19 https://doi.org/10.1016/0006-8993(88)90835-9
20 https://doi.org/10.1016/j.expneurol.2012.07.013
21 https://doi.org/10.1016/j.gene.2014.09.046
22 https://doi.org/10.1016/j.mcn.2012.07.003
23 https://doi.org/10.1016/j.nbd.2012.01.013
24 https://doi.org/10.1016/j.nbd.2014.02.012
25 https://doi.org/10.1016/j.nbd.2015.08.004
26 https://doi.org/10.1016/j.ymgme.2014.05.009
27 https://doi.org/10.1016/s0896-6273(02)00670-0
28 https://doi.org/10.1038/jcbfm.2013.64
29 https://doi.org/10.1038/mt.2015.145
30 https://doi.org/10.1042/an20110019
31 https://doi.org/10.1046/j.1471-4159.1996.66031118.x
32 https://doi.org/10.1093/brain/103.3.695
33 https://doi.org/10.1093/brain/39.1-2.74
34 https://doi.org/10.1093/brain/awt215
35 https://doi.org/10.1093/hmg/ddv086
36 https://doi.org/10.1093/nar/19.15.4293
37 https://doi.org/10.1146/annurev.neuro.051508.135722
38 https://doi.org/10.1371/journal.pone.0026164
39 https://doi.org/10.1523/jneurosci.0217-13.2013
40 https://doi.org/10.1523/jneurosci.1753-09.2009
41 https://doi.org/10.1523/jneurosci.2431-14.2015
42 https://doi.org/10.1523/jneurosci.3095-15.2016
43 https://doi.org/10.1523/jneurosci.4199-14.2015
44 https://doi.org/10.1523/jneurosci.5597-08.2009
45 https://doi.org/10.15252/emmm.201505850
46 schema:datePublished 2016-12
47 schema:datePublishedReg 2016-12-01
48 schema:description Krabbe disease (KD) is a neurodegenerative disorder caused by the lack of β- galactosylceramidase enzymatic activity and by widespread accumulation of the cytotoxic galactosyl-sphingosine in neuronal, myelinating and endothelial cells. Despite the wide use of Twitcher mice as experimental model for KD, the ultrastructure of this model is partial and mainly addressing peripheral nerves. More details are requested to elucidate the basis of the motor defects, which are the first to appear during KD onset. Here we use transmission electron microscopy (TEM) to focus on the alterations produced by KD in the lower motor system at postnatal day 15 (P15), a nearly asymptomatic stage, and in the juvenile P30 mouse. We find mild effects on motorneuron soma, severe ones on sciatic nerves and very severe effects on nerve terminals and neuromuscular junctions at P30, with peripheral damage being already detectable at P15. Finally, we find that the gastrocnemius muscle undergoes atrophy and structural changes that are independent of denervation at P15. Our data further characterize the ultrastructural analysis of the KD mouse model, and support recent theories of a dying-back mechanism for neuronal degeneration, which is independent of demyelination.
49 schema:genre research_article
50 schema:inLanguage en
51 schema:isAccessibleForFree true
52 schema:isPartOf N84b7ae95c1ed40bfa419a3d9d987da08
53 N9559dfaf858e48d09a016d9abc99a959
54 sg:journal.1045337
55 schema:name Ultrastructural Characterization of the Lower Motor System in a Mouse Model of Krabbe Disease
56 schema:pagination 1
57 schema:productId N014a11ef8f4b4d34b8bed964d27d6321
58 N1ef8ea7d7cbb48b693308b404201d5b9
59 Nee09bb53ea92467b99f0be9476815bc2
60 schema:sameAs https://app.dimensions.ai/details/publication/pub.1056467545
61 https://doi.org/10.1038/s41598-016-0001-8
62 schema:sdDatePublished 2019-04-10T20:35
63 schema:sdLicense https://scigraph.springernature.com/explorer/license/
64 schema:sdPublisher Ncd32422fae894c30b6304760617e3ac1
65 schema:url http://www.nature.com/articles/s41598-016-0001-8
66 sgo:license sg:explorer/license/
67 sgo:sdDataset articles
68 rdf:type schema:ScholarlyArticle
69 N014a11ef8f4b4d34b8bed964d27d6321 schema:name doi
70 schema:value 10.1038/s41598-016-0001-8
71 rdf:type schema:PropertyValue
72 N0364e77674a44558a317e7b8155294c9 schema:name Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy
73 rdf:type schema:Organization
74 N1ef8ea7d7cbb48b693308b404201d5b9 schema:name readcube_id
75 schema:value ca46b7f85d1ce1378225471d9d461dccd0e1bece53b300b9ac0b890809838187
76 rdf:type schema:PropertyValue
77 N3424b2601dce47be9d9578022f9a0cc6 schema:name Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy
78 rdf:type schema:Organization
79 N50b3a2893d3a4d169ba34c75f4d0aa0f schema:name NEST, Istituto Nanoscienze-CNR, Pisa, Italy
80 rdf:type schema:Organization
81 N53ddc41adb754059817ec3de90769e5d rdf:first sg:person.01100741224.17
82 rdf:rest N92d002520ec947c5a0dcdc71827955d7
83 N57be04ec7b37478fbf99e203ececcde7 rdf:first sg:person.0676657141.49
84 rdf:rest N5b0de481dc3a474cbf96de5fc9f09877
85 N5b0de481dc3a474cbf96de5fc9f09877 rdf:first sg:person.01352275273.03
86 rdf:rest N53ddc41adb754059817ec3de90769e5d
87 N80cd592d3ee845a29f6aabdac504273a rdf:first sg:person.0632662545.29
88 rdf:rest Nd6966020452a49b08dcd569640afb768
89 N84b7ae95c1ed40bfa419a3d9d987da08 schema:volumeNumber 6
90 rdf:type schema:PublicationVolume
91 N92d002520ec947c5a0dcdc71827955d7 rdf:first sg:person.0601671705.59
92 rdf:rest N80cd592d3ee845a29f6aabdac504273a
93 N9559dfaf858e48d09a016d9abc99a959 schema:issueNumber 1
94 rdf:type schema:PublicationIssue
95 Ncd32422fae894c30b6304760617e3ac1 schema:name Springer Nature - SN SciGraph project
96 rdf:type schema:Organization
97 Nd2f02c8078384814bbec4ca9554f3ab1 rdf:first sg:person.0704765127.66
98 rdf:rest N57be04ec7b37478fbf99e203ececcde7
99 Nd6966020452a49b08dcd569640afb768 rdf:first sg:person.01331013067.25
100 rdf:rest Nde29db39dde74870aea435d1df6a7319
101 Nde29db39dde74870aea435d1df6a7319 rdf:first sg:person.0741334155.71
102 rdf:rest rdf:nil
103 Necab79f8f7f74aaea160a711692e336f schema:name Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy
104 rdf:type schema:Organization
105 Nee09bb53ea92467b99f0be9476815bc2 schema:name dimensions_id
106 schema:value pub.1056467545
107 rdf:type schema:PropertyValue
108 Nf9e655746a9745089b861b0b99696e85 schema:name Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy
109 rdf:type schema:Organization
110 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
111 schema:name Medical and Health Sciences
112 rdf:type schema:DefinedTerm
113 anzsrc-for:1109 schema:inDefinedTermSet anzsrc-for:
114 schema:name Neurosciences
115 rdf:type schema:DefinedTerm
116 sg:journal.1045337 schema:issn 2045-2322
117 schema:name Scientific Reports
118 rdf:type schema:Periodical
119 sg:person.01100741224.17 schema:affiliation N50b3a2893d3a4d169ba34c75f4d0aa0f
120 schema:familyName Landi
121 schema:givenName Silvia
122 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01100741224.17
123 rdf:type schema:Person
124 sg:person.01331013067.25 schema:affiliation Necab79f8f7f74aaea160a711692e336f
125 schema:familyName Piazza
126 schema:givenName Vincenzo
127 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01331013067.25
128 rdf:type schema:Person
129 sg:person.01352275273.03 schema:affiliation https://www.grid.ac/institutes/grid.6093.c
130 schema:familyName Parlanti
131 schema:givenName Paola
132 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01352275273.03
133 rdf:type schema:Person
134 sg:person.0601671705.59 schema:affiliation https://www.grid.ac/institutes/grid.478935.4
135 schema:familyName Tonazzini
136 schema:givenName Ilaria
137 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0601671705.59
138 rdf:type schema:Person
139 sg:person.0632662545.29 schema:affiliation https://www.grid.ac/institutes/grid.6093.c
140 schema:familyName Cecchini
141 schema:givenName Marco
142 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0632662545.29
143 rdf:type schema:Person
144 sg:person.0676657141.49 schema:affiliation N0364e77674a44558a317e7b8155294c9
145 schema:familyName Marchetti
146 schema:givenName Laura
147 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0676657141.49
148 rdf:type schema:Person
149 sg:person.0704765127.66 schema:affiliation Nf9e655746a9745089b861b0b99696e85
150 schema:familyName Cappello
151 schema:givenName Valentina
152 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0704765127.66
153 rdf:type schema:Person
154 sg:person.0741334155.71 schema:affiliation N3424b2601dce47be9d9578022f9a0cc6
155 schema:familyName Gemmi
156 schema:givenName Mauro
157 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0741334155.71
158 rdf:type schema:Person
159 sg:pub.10.1007/s00401-011-0814-2 schema:sameAs https://app.dimensions.ai/details/publication/pub.1049873905
160 https://doi.org/10.1007/s00401-011-0814-2
161 rdf:type schema:CreativeWork
162 sg:pub.10.1007/s13311-011-0080-y schema:sameAs https://app.dimensions.ai/details/publication/pub.1010548540
163 https://doi.org/10.1007/s13311-011-0080-y
164 rdf:type schema:CreativeWork
165 sg:pub.10.1023/b:neur.0000020628.36013.88 schema:sameAs https://app.dimensions.ai/details/publication/pub.1014276565
166 https://doi.org/10.1023/b:neur.0000020628.36013.88
167 rdf:type schema:CreativeWork
168 sg:pub.10.1038/cddis.2014.483 schema:sameAs https://app.dimensions.ai/details/publication/pub.1005725448
169 https://doi.org/10.1038/cddis.2014.483
170 rdf:type schema:CreativeWork
171 sg:pub.10.1038/nn.3281 schema:sameAs https://app.dimensions.ai/details/publication/pub.1042231649
172 https://doi.org/10.1038/nn.3281
173 rdf:type schema:CreativeWork
174 sg:pub.10.1038/nn1653 schema:sameAs https://app.dimensions.ai/details/publication/pub.1010416853
175 https://doi.org/10.1038/nn1653
176 rdf:type schema:CreativeWork
177 sg:pub.10.1038/srep00045 schema:sameAs https://app.dimensions.ai/details/publication/pub.1005632431
178 https://doi.org/10.1038/srep00045
179 rdf:type schema:CreativeWork
180 sg:pub.10.1186/s13395-014-0022-6 schema:sameAs https://app.dimensions.ai/details/publication/pub.1034682712
181 https://doi.org/10.1186/s13395-014-0022-6
182 rdf:type schema:CreativeWork
183 https://app.dimensions.ai/details/publication/pub.1079702738 schema:CreativeWork
184 https://app.dimensions.ai/details/publication/pub.1081997437 schema:CreativeWork
185 https://doi.org/10.1002/(sici)1098-1004(1997)10:4<268::aid-humu2>3.0.co;2-d schema:sameAs https://app.dimensions.ai/details/publication/pub.1013204335
186 rdf:type schema:CreativeWork
187 https://doi.org/10.1002/glia.20761 schema:sameAs https://app.dimensions.ai/details/publication/pub.1017134781
188 rdf:type schema:CreativeWork
189 https://doi.org/10.1002/glia.20778 schema:sameAs https://app.dimensions.ai/details/publication/pub.1046916402
190 rdf:type schema:CreativeWork
191 https://doi.org/10.1002/jbio.201500305 schema:sameAs https://app.dimensions.ai/details/publication/pub.1009600906
192 rdf:type schema:CreativeWork
193 https://doi.org/10.1002/jnr.23744 schema:sameAs https://app.dimensions.ai/details/publication/pub.1052425771
194 rdf:type schema:CreativeWork
195 https://doi.org/10.1016/0006-8993(88)90835-9 schema:sameAs https://app.dimensions.ai/details/publication/pub.1000076080
196 rdf:type schema:CreativeWork
197 https://doi.org/10.1016/j.expneurol.2012.07.013 schema:sameAs https://app.dimensions.ai/details/publication/pub.1013420690
198 rdf:type schema:CreativeWork
199 https://doi.org/10.1016/j.gene.2014.09.046 schema:sameAs https://app.dimensions.ai/details/publication/pub.1016509648
200 rdf:type schema:CreativeWork
201 https://doi.org/10.1016/j.mcn.2012.07.003 schema:sameAs https://app.dimensions.ai/details/publication/pub.1051714196
202 rdf:type schema:CreativeWork
203 https://doi.org/10.1016/j.nbd.2012.01.013 schema:sameAs https://app.dimensions.ai/details/publication/pub.1005580237
204 rdf:type schema:CreativeWork
205 https://doi.org/10.1016/j.nbd.2014.02.012 schema:sameAs https://app.dimensions.ai/details/publication/pub.1017740527
206 rdf:type schema:CreativeWork
207 https://doi.org/10.1016/j.nbd.2015.08.004 schema:sameAs https://app.dimensions.ai/details/publication/pub.1005813908
208 rdf:type schema:CreativeWork
209 https://doi.org/10.1016/j.ymgme.2014.05.009 schema:sameAs https://app.dimensions.ai/details/publication/pub.1045807891
210 rdf:type schema:CreativeWork
211 https://doi.org/10.1016/s0896-6273(02)00670-0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1030361991
212 rdf:type schema:CreativeWork
213 https://doi.org/10.1038/jcbfm.2013.64 schema:sameAs https://app.dimensions.ai/details/publication/pub.1048484759
214 rdf:type schema:CreativeWork
215 https://doi.org/10.1038/mt.2015.145 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029771913
216 rdf:type schema:CreativeWork
217 https://doi.org/10.1042/an20110019 schema:sameAs https://app.dimensions.ai/details/publication/pub.1018677978
218 rdf:type schema:CreativeWork
219 https://doi.org/10.1046/j.1471-4159.1996.66031118.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1007273365
220 rdf:type schema:CreativeWork
221 https://doi.org/10.1093/brain/103.3.695 schema:sameAs https://app.dimensions.ai/details/publication/pub.1059437410
222 rdf:type schema:CreativeWork
223 https://doi.org/10.1093/brain/39.1-2.74 schema:sameAs https://app.dimensions.ai/details/publication/pub.1059440791
224 rdf:type schema:CreativeWork
225 https://doi.org/10.1093/brain/awt215 schema:sameAs https://app.dimensions.ai/details/publication/pub.1016427155
226 rdf:type schema:CreativeWork
227 https://doi.org/10.1093/hmg/ddv086 schema:sameAs https://app.dimensions.ai/details/publication/pub.1033869737
228 rdf:type schema:CreativeWork
229 https://doi.org/10.1093/nar/19.15.4293 schema:sameAs https://app.dimensions.ai/details/publication/pub.1034171019
230 rdf:type schema:CreativeWork
231 https://doi.org/10.1146/annurev.neuro.051508.135722 schema:sameAs https://app.dimensions.ai/details/publication/pub.1020995537
232 rdf:type schema:CreativeWork
233 https://doi.org/10.1371/journal.pone.0026164 schema:sameAs https://app.dimensions.ai/details/publication/pub.1043971475
234 rdf:type schema:CreativeWork
235 https://doi.org/10.1523/jneurosci.0217-13.2013 schema:sameAs https://app.dimensions.ai/details/publication/pub.1043478470
236 rdf:type schema:CreativeWork
237 https://doi.org/10.1523/jneurosci.1753-09.2009 schema:sameAs https://app.dimensions.ai/details/publication/pub.1017032597
238 rdf:type schema:CreativeWork
239 https://doi.org/10.1523/jneurosci.2431-14.2015 schema:sameAs https://app.dimensions.ai/details/publication/pub.1051295323
240 rdf:type schema:CreativeWork
241 https://doi.org/10.1523/jneurosci.3095-15.2016 schema:sameAs https://app.dimensions.ai/details/publication/pub.1038619602
242 rdf:type schema:CreativeWork
243 https://doi.org/10.1523/jneurosci.4199-14.2015 schema:sameAs https://app.dimensions.ai/details/publication/pub.1033568851
244 rdf:type schema:CreativeWork
245 https://doi.org/10.1523/jneurosci.5597-08.2009 schema:sameAs https://app.dimensions.ai/details/publication/pub.1015262280
246 rdf:type schema:CreativeWork
247 https://doi.org/10.15252/emmm.201505850 schema:sameAs https://app.dimensions.ai/details/publication/pub.1010545031
248 rdf:type schema:CreativeWork
249 https://www.grid.ac/institutes/grid.478935.4 schema:alternateName Fondazione Umberto Veronesi
250 schema:name Fondazione Umberto Veronesi, Milano, Italy
251 NEST, Istituto Nanoscienze-CNR, Pisa, Italy
252 rdf:type schema:Organization
253 https://www.grid.ac/institutes/grid.6093.c schema:alternateName Scuola Normale Superiore di Pisa
254 schema:name Center for Nanotechnology Innovation@NEST, Istituto Italiano di Tecnologia Piazza San Silvestro, 12, Pisa, Italy
255 NEST, Istituto Nanoscienze-CNR, Pisa, Italy
256 NEST, Scuola Normale Superiore, Pisa, Italy
257 rdf:type schema:Organization
 




Preview window. Press ESC to close (or click here)


...