ALS-associated mutant FUS inhibits macroautophagy which is restored by overexpression of Rab1 View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2015-12

AUTHORS

K Y Soo, J Sultana, AE King, RAK Atkinson, S T Warraich, V Sundaramoorthy, I Blair, M A Farg, J D Atkin

ABSTRACT

Amyotrophic lateral sclerosis (ALS) is characterised by the formation of intracellular misfolded protein inclusions that form in motor neurons. Autophagy is the major degradation pathway for aggregate-prone proteins within lysosomes. Autophagy begins by the production of the omegasome, forming the autophagosome membrane, which then fuses with the lysosome. Mutations in fused in sarcoma (FUS) cause 5% of familial ALS cases and FUS-positive inclusions are also formed in sporadic ALS tissues. In this study, we demonstrate that the expression of ALS-associated mutant FUS impairs autophagy in neuronal cells. In mutant FUS-expressing neuronal cells, accumulation of ubiquitinated proteins and autophagy substrates p62 and NBR1 was detected, and formation of both the omegasome and autophagosome was inhibited in these cells. However, overexpression of Rab1 rescued these defects, suggesting that Rab1 is protective in ALS. The number of LC3-positive vesicles was also increased in motor neurons from the spinal cord of an ALS patient carrying a FUS (R521C) mutation compared with a control patient, providing additional evidence that autophagy is dysregulated in mutant FUS-associated ALS. This study provides further understanding of the intricate autophagy system and neurodegeneration in ALS. More... »

PAGES

15030

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/cddiscovery.2015.30

DOI

http://dx.doi.org/10.1038/cddiscovery.2015.30

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1000744579

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/27551461


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": {
          "alternateName": "La Trobe University", 
          "id": "https://www.grid.ac/institutes/grid.1018.8", 
          "name": [
            "Department of Biochemistry and Genetics, La Trobe Institute for Molecular Science, La Trobe University, Bundoora, Victoria, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Soo", 
        "givenName": "K Y", 
        "id": "sg:person.01207106043.08", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01207106043.08"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Macquarie University", 
          "id": "https://www.grid.ac/institutes/grid.1004.5", 
          "name": [
            "Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Sultana", 
        "givenName": "J", 
        "id": "sg:person.01107036032.14", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01107036032.14"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of Tasmania", 
          "id": "https://www.grid.ac/institutes/grid.1009.8", 
          "name": [
            "Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, Tasmania, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "King", 
        "givenName": "AE", 
        "id": "sg:person.013510241632.98", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.013510241632.98"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "University of Tasmania", 
          "id": "https://www.grid.ac/institutes/grid.1009.8", 
          "name": [
            "Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, Tasmania, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Atkinson", 
        "givenName": "RAK", 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Macquarie University", 
          "id": "https://www.grid.ac/institutes/grid.1004.5", 
          "name": [
            "Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Warraich", 
        "givenName": "S T", 
        "id": "sg:person.01267714255.75", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01267714255.75"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Macquarie University", 
          "id": "https://www.grid.ac/institutes/grid.1004.5", 
          "name": [
            "Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Sundaramoorthy", 
        "givenName": "V", 
        "id": "sg:person.01073105221.87", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01073105221.87"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Macquarie University", 
          "id": "https://www.grid.ac/institutes/grid.1004.5", 
          "name": [
            "Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Blair", 
        "givenName": "I", 
        "id": "sg:person.01043100355.97", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01043100355.97"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "La Trobe University", 
          "id": "https://www.grid.ac/institutes/grid.1018.8", 
          "name": [
            "Department of Biochemistry and Genetics, La Trobe Institute for Molecular Science, La Trobe University, Bundoora, Victoria, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Farg", 
        "givenName": "M A", 
        "id": "sg:person.0643343176.90", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0643343176.90"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Macquarie University", 
          "id": "https://www.grid.ac/institutes/grid.1004.5", 
          "name": [
            "Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Atkin", 
        "givenName": "J D", 
        "id": "sg:person.01255221243.24", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01255221243.24"
        ], 
        "type": "Person"
      }
    ], 
    "citation": [
      {
        "id": "https://doi.org/10.1111/j.1600-0854.2010.01086.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001334821"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/j.1600-0854.2010.01086.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001334821"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.cell.2009.03.048", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001834748"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.neulet.2009.11.055", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1002574965"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/brain/awt030", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1006306003"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.neurobiolaging.2014.07.026", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1007269184"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nrm3249", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1007855841", 
          "https://doi.org/10.1038/nrm3249"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1093/nar/gkq837", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1008818732"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1001/archneurol.2010.52", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1008955251"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/cdd.2013.187", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1009579079", 
          "https://doi.org/10.1038/cdd.2013.187"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1136/jmg.36.10.739", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1009808839"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.cell.2007.10.035", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1011978647"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1074/jbc.m405988200", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1012141205"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.cell.2013.05.037", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1012347412"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.brainres.2007.06.045", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1012507337"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.molcel.2009.01.021", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1014194959"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/neup.12058", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016101043"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.neulet.2012.10.003", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1017806747"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1242/jcs.03172", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1019146455"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1083/jcb.200803137", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1020971115"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1083/jcb.125.2.225", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1022398138"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.febslet.2011.01.045", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1025161672"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nsmb.2163", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1025911101", 
          "https://doi.org/10.1038/nsmb.2163"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.19496", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1026175635"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.19496", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1026175635"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0092-8674(86)90597-0", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1026299955"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1083/jcb.201202061", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1026456811"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/nen.0b013e3182160690", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1027579372"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.14814/phy2.12271", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1028569388"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1091/mbc.12.11.3690", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1029254724"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1126/science.1165942", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1030298206"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1186/2051-5960-1-59", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1032216362", 
          "https://doi.org/10.1186/2051-5960-1-59"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1083/jcb.201302044", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1033173546"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/j.1440-1789.2010.01129.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1033296861"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1074/jbc.m702824200", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1040304237"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/nature10353", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1041330211", 
          "https://doi.org/10.1038/nature10353"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1155/2012/189794", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1042508093"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1038/embor.2008.238", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1043164473"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s10571-010-9507-y", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044025346", 
          "https://doi.org/10.1007/s10571-010-9507-y"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s10571-010-9507-y", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044025346", 
          "https://doi.org/10.1007/s10571-010-9507-y"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.neurobiolaging.2012.02.009", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044071686"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1126/science.1166066", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044437162"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1136/jnnp.2009.194399", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1044746706"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1083/jcb.201003122", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1046751612"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1074/jbc.m603337200", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1048312989"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1074/jbc.m111.333450", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1050921545"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1038/emboj.2012.261", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1053326953"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1038/ncb2848", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1053370412", 
          "https://doi.org/10.1038/ncb2848"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.18632/aging.100385", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1068670695"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.26917", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072289899"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.3419", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072290120"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.5.5.8566", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072290409"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.5524", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072290547"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.7.4.14541", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072291013"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/auto.8840", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1072291173"
        ], 
        "type": "CreativeWork"
      }
    ], 
    "datePublished": "2015-12", 
    "datePublishedReg": "2015-12-01", 
    "description": "Amyotrophic lateral sclerosis (ALS) is characterised by the formation of intracellular misfolded protein inclusions that form in motor neurons. Autophagy is the major degradation pathway for aggregate-prone proteins within lysosomes. Autophagy begins by the production of the omegasome, forming the autophagosome membrane, which then fuses with the lysosome. Mutations in fused in sarcoma (FUS) cause 5% of familial ALS cases and FUS-positive inclusions are also formed in sporadic ALS tissues. In this study, we demonstrate that the expression of ALS-associated mutant FUS impairs autophagy in neuronal cells. In mutant FUS-expressing neuronal cells, accumulation of ubiquitinated proteins and autophagy substrates p62 and NBR1 was detected, and formation of both the omegasome and autophagosome was inhibited in these cells. However, overexpression of Rab1 rescued these defects, suggesting that Rab1 is protective in ALS. The number of LC3-positive vesicles was also increased in motor neurons from the spinal cord of an ALS patient carrying a FUS (R521C) mutation compared with a control patient, providing additional evidence that autophagy is dysregulated in mutant FUS-associated ALS. This study provides further understanding of the intricate autophagy system and neurodegeneration in ALS. ", 
    "genre": "research_article", 
    "id": "sg:pub.10.1038/cddiscovery.2015.30", 
    "inLanguage": [
      "en"
    ], 
    "isAccessibleForFree": true, 
    "isFundedItemOf": [
      {
        "id": "sg:grant.6733786", 
        "type": "MonetaryGrant"
      }, 
      {
        "id": "sg:grant.6734366", 
        "type": "MonetaryGrant"
      }
    ], 
    "isPartOf": [
      {
        "id": "sg:journal.1052270", 
        "issn": [
          "2058-7716"
        ], 
        "name": "Cell Death Discovery", 
        "type": "Periodical"
      }, 
      {
        "issueNumber": "1", 
        "type": "PublicationIssue"
      }, 
      {
        "type": "PublicationVolume", 
        "volumeNumber": "1"
      }
    ], 
    "name": "ALS-associated mutant FUS inhibits macroautophagy which is restored by overexpression of Rab1", 
    "pagination": "15030", 
    "productId": [
      {
        "name": "readcube_id", 
        "type": "PropertyValue", 
        "value": [
          "038bc009ae1a83034bb8f90ef02a89cefb1ef114ac7adcf01974d39c758b8c9a"
        ]
      }, 
      {
        "name": "pubmed_id", 
        "type": "PropertyValue", 
        "value": [
          "27551461"
        ]
      }, 
      {
        "name": "nlm_unique_id", 
        "type": "PropertyValue", 
        "value": [
          "101665035"
        ]
      }, 
      {
        "name": "doi", 
        "type": "PropertyValue", 
        "value": [
          "10.1038/cddiscovery.2015.30"
        ]
      }, 
      {
        "name": "dimensions_id", 
        "type": "PropertyValue", 
        "value": [
          "pub.1000744579"
        ]
      }
    ], 
    "sameAs": [
      "https://doi.org/10.1038/cddiscovery.2015.30", 
      "https://app.dimensions.ai/details/publication/pub.1000744579"
    ], 
    "sdDataset": "articles", 
    "sdDatePublished": "2019-04-10T19:15", 
    "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_8678_00000549.jsonl", 
    "type": "ScholarlyArticle", 
    "url": "http://www.nature.com/articles/cddiscovery201530"
  }
]
 

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/cddiscovery.2015.30'

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/cddiscovery.2015.30'

Turtle is a human-readable linked data format.

curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1038/cddiscovery.2015.30'

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

curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1038/cddiscovery.2015.30'


 

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

296 TRIPLES      21 PREDICATES      81 URIs      21 LITERALS      9 BLANK NODES

Subject Predicate Object
1 sg:pub.10.1038/cddiscovery.2015.30 schema:about anzsrc-for:11
2 anzsrc-for:1109
3 schema:author N50f15707e39b4a10ad17bd5dfe2c2713
4 schema:citation sg:pub.10.1007/s10571-010-9507-y
5 sg:pub.10.1038/cdd.2013.187
6 sg:pub.10.1038/nature10353
7 sg:pub.10.1038/ncb2848
8 sg:pub.10.1038/nrm3249
9 sg:pub.10.1038/nsmb.2163
10 sg:pub.10.1186/2051-5960-1-59
11 https://doi.org/10.1001/archneurol.2010.52
12 https://doi.org/10.1016/0092-8674(86)90597-0
13 https://doi.org/10.1016/j.brainres.2007.06.045
14 https://doi.org/10.1016/j.cell.2007.10.035
15 https://doi.org/10.1016/j.cell.2009.03.048
16 https://doi.org/10.1016/j.cell.2013.05.037
17 https://doi.org/10.1016/j.febslet.2011.01.045
18 https://doi.org/10.1016/j.molcel.2009.01.021
19 https://doi.org/10.1016/j.neulet.2009.11.055
20 https://doi.org/10.1016/j.neulet.2012.10.003
21 https://doi.org/10.1016/j.neurobiolaging.2012.02.009
22 https://doi.org/10.1016/j.neurobiolaging.2014.07.026
23 https://doi.org/10.1038/emboj.2012.261
24 https://doi.org/10.1038/embor.2008.238
25 https://doi.org/10.1074/jbc.m111.333450
26 https://doi.org/10.1074/jbc.m405988200
27 https://doi.org/10.1074/jbc.m603337200
28 https://doi.org/10.1074/jbc.m702824200
29 https://doi.org/10.1083/jcb.125.2.225
30 https://doi.org/10.1083/jcb.200803137
31 https://doi.org/10.1083/jcb.201003122
32 https://doi.org/10.1083/jcb.201202061
33 https://doi.org/10.1083/jcb.201302044
34 https://doi.org/10.1091/mbc.12.11.3690
35 https://doi.org/10.1093/brain/awt030
36 https://doi.org/10.1093/nar/gkq837
37 https://doi.org/10.1097/nen.0b013e3182160690
38 https://doi.org/10.1111/j.1440-1789.2010.01129.x
39 https://doi.org/10.1111/j.1600-0854.2010.01086.x
40 https://doi.org/10.1111/neup.12058
41 https://doi.org/10.1126/science.1165942
42 https://doi.org/10.1126/science.1166066
43 https://doi.org/10.1136/jmg.36.10.739
44 https://doi.org/10.1136/jnnp.2009.194399
45 https://doi.org/10.1155/2012/189794
46 https://doi.org/10.1242/jcs.03172
47 https://doi.org/10.14814/phy2.12271
48 https://doi.org/10.18632/aging.100385
49 https://doi.org/10.4161/auto.19496
50 https://doi.org/10.4161/auto.26917
51 https://doi.org/10.4161/auto.3419
52 https://doi.org/10.4161/auto.5.5.8566
53 https://doi.org/10.4161/auto.5524
54 https://doi.org/10.4161/auto.7.4.14541
55 https://doi.org/10.4161/auto.8840
56 schema:datePublished 2015-12
57 schema:datePublishedReg 2015-12-01
58 schema:description Amyotrophic lateral sclerosis (ALS) is characterised by the formation of intracellular misfolded protein inclusions that form in motor neurons. Autophagy is the major degradation pathway for aggregate-prone proteins within lysosomes. Autophagy begins by the production of the omegasome, forming the autophagosome membrane, which then fuses with the lysosome. Mutations in fused in sarcoma (FUS) cause 5% of familial ALS cases and FUS-positive inclusions are also formed in sporadic ALS tissues. In this study, we demonstrate that the expression of ALS-associated mutant FUS impairs autophagy in neuronal cells. In mutant FUS-expressing neuronal cells, accumulation of ubiquitinated proteins and autophagy substrates p62 and NBR1 was detected, and formation of both the omegasome and autophagosome was inhibited in these cells. However, overexpression of Rab1 rescued these defects, suggesting that Rab1 is protective in ALS. The number of LC3-positive vesicles was also increased in motor neurons from the spinal cord of an ALS patient carrying a FUS (R521C) mutation compared with a control patient, providing additional evidence that autophagy is dysregulated in mutant FUS-associated ALS. This study provides further understanding of the intricate autophagy system and neurodegeneration in ALS.
59 schema:genre research_article
60 schema:inLanguage en
61 schema:isAccessibleForFree true
62 schema:isPartOf Nbd87918888d5442bb627b31341c3f52a
63 Ndf5ce0376482436ab162dfac7eb3b27b
64 sg:journal.1052270
65 schema:name ALS-associated mutant FUS inhibits macroautophagy which is restored by overexpression of Rab1
66 schema:pagination 15030
67 schema:productId N16e81a7adccc4efca52f26c4d2efea99
68 N701e8d8230b94649b8fa1634e99a9cc3
69 N835e9b215aa745eebfdabbcff22d7b6f
70 Ncc25ff28184140678fa8061a64a52327
71 Nefd274a6ea1945009135981b43d77e95
72 schema:sameAs https://app.dimensions.ai/details/publication/pub.1000744579
73 https://doi.org/10.1038/cddiscovery.2015.30
74 schema:sdDatePublished 2019-04-10T19:15
75 schema:sdLicense https://scigraph.springernature.com/explorer/license/
76 schema:sdPublisher Nb52527b764a6473c8694b2e8eb2840be
77 schema:url http://www.nature.com/articles/cddiscovery201530
78 sgo:license sg:explorer/license/
79 sgo:sdDataset articles
80 rdf:type schema:ScholarlyArticle
81 N16e81a7adccc4efca52f26c4d2efea99 schema:name nlm_unique_id
82 schema:value 101665035
83 rdf:type schema:PropertyValue
84 N50f15707e39b4a10ad17bd5dfe2c2713 rdf:first sg:person.01207106043.08
85 rdf:rest N8c1fe03e074d4f09ab44cce07669b15f
86 N701e8d8230b94649b8fa1634e99a9cc3 schema:name readcube_id
87 schema:value 038bc009ae1a83034bb8f90ef02a89cefb1ef114ac7adcf01974d39c758b8c9a
88 rdf:type schema:PropertyValue
89 N81910ac58b1745f88ae922324b471690 rdf:first sg:person.01073105221.87
90 rdf:rest Nd1103e95b7eb4ae9a2fa978fe2d4daab
91 N835e9b215aa745eebfdabbcff22d7b6f schema:name doi
92 schema:value 10.1038/cddiscovery.2015.30
93 rdf:type schema:PropertyValue
94 N8b0670caaa034ab696e48f5d89e402c0 rdf:first sg:person.013510241632.98
95 rdf:rest Nc22bd760f7574de6bb477744149e0ffa
96 N8be485b3fc7d4f38a975a836e4a3ef6e rdf:first sg:person.0643343176.90
97 rdf:rest Nf579ed82a5b4498e8f485fac54d7de64
98 N8c1fe03e074d4f09ab44cce07669b15f rdf:first sg:person.01107036032.14
99 rdf:rest N8b0670caaa034ab696e48f5d89e402c0
100 N9224c62242ac45da95cdc3fa9cf629e7 rdf:first sg:person.01267714255.75
101 rdf:rest N81910ac58b1745f88ae922324b471690
102 Nb11adc97caa34f9a9878ea19b442e03b schema:affiliation https://www.grid.ac/institutes/grid.1009.8
103 schema:familyName Atkinson
104 schema:givenName RAK
105 rdf:type schema:Person
106 Nb52527b764a6473c8694b2e8eb2840be schema:name Springer Nature - SN SciGraph project
107 rdf:type schema:Organization
108 Nbd87918888d5442bb627b31341c3f52a schema:volumeNumber 1
109 rdf:type schema:PublicationVolume
110 Nc22bd760f7574de6bb477744149e0ffa rdf:first Nb11adc97caa34f9a9878ea19b442e03b
111 rdf:rest N9224c62242ac45da95cdc3fa9cf629e7
112 Ncc25ff28184140678fa8061a64a52327 schema:name dimensions_id
113 schema:value pub.1000744579
114 rdf:type schema:PropertyValue
115 Nd1103e95b7eb4ae9a2fa978fe2d4daab rdf:first sg:person.01043100355.97
116 rdf:rest N8be485b3fc7d4f38a975a836e4a3ef6e
117 Ndf5ce0376482436ab162dfac7eb3b27b schema:issueNumber 1
118 rdf:type schema:PublicationIssue
119 Nefd274a6ea1945009135981b43d77e95 schema:name pubmed_id
120 schema:value 27551461
121 rdf:type schema:PropertyValue
122 Nf579ed82a5b4498e8f485fac54d7de64 rdf:first sg:person.01255221243.24
123 rdf:rest rdf:nil
124 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
125 schema:name Medical and Health Sciences
126 rdf:type schema:DefinedTerm
127 anzsrc-for:1109 schema:inDefinedTermSet anzsrc-for:
128 schema:name Neurosciences
129 rdf:type schema:DefinedTerm
130 sg:grant.6733786 http://pending.schema.org/fundedItem sg:pub.10.1038/cddiscovery.2015.30
131 rdf:type schema:MonetaryGrant
132 sg:grant.6734366 http://pending.schema.org/fundedItem sg:pub.10.1038/cddiscovery.2015.30
133 rdf:type schema:MonetaryGrant
134 sg:journal.1052270 schema:issn 2058-7716
135 schema:name Cell Death Discovery
136 rdf:type schema:Periodical
137 sg:person.01043100355.97 schema:affiliation https://www.grid.ac/institutes/grid.1004.5
138 schema:familyName Blair
139 schema:givenName I
140 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01043100355.97
141 rdf:type schema:Person
142 sg:person.01073105221.87 schema:affiliation https://www.grid.ac/institutes/grid.1004.5
143 schema:familyName Sundaramoorthy
144 schema:givenName V
145 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01073105221.87
146 rdf:type schema:Person
147 sg:person.01107036032.14 schema:affiliation https://www.grid.ac/institutes/grid.1004.5
148 schema:familyName Sultana
149 schema:givenName J
150 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01107036032.14
151 rdf:type schema:Person
152 sg:person.01207106043.08 schema:affiliation https://www.grid.ac/institutes/grid.1018.8
153 schema:familyName Soo
154 schema:givenName K Y
155 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01207106043.08
156 rdf:type schema:Person
157 sg:person.01255221243.24 schema:affiliation https://www.grid.ac/institutes/grid.1004.5
158 schema:familyName Atkin
159 schema:givenName J D
160 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01255221243.24
161 rdf:type schema:Person
162 sg:person.01267714255.75 schema:affiliation https://www.grid.ac/institutes/grid.1004.5
163 schema:familyName Warraich
164 schema:givenName S T
165 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01267714255.75
166 rdf:type schema:Person
167 sg:person.013510241632.98 schema:affiliation https://www.grid.ac/institutes/grid.1009.8
168 schema:familyName King
169 schema:givenName AE
170 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.013510241632.98
171 rdf:type schema:Person
172 sg:person.0643343176.90 schema:affiliation https://www.grid.ac/institutes/grid.1018.8
173 schema:familyName Farg
174 schema:givenName M A
175 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0643343176.90
176 rdf:type schema:Person
177 sg:pub.10.1007/s10571-010-9507-y schema:sameAs https://app.dimensions.ai/details/publication/pub.1044025346
178 https://doi.org/10.1007/s10571-010-9507-y
179 rdf:type schema:CreativeWork
180 sg:pub.10.1038/cdd.2013.187 schema:sameAs https://app.dimensions.ai/details/publication/pub.1009579079
181 https://doi.org/10.1038/cdd.2013.187
182 rdf:type schema:CreativeWork
183 sg:pub.10.1038/nature10353 schema:sameAs https://app.dimensions.ai/details/publication/pub.1041330211
184 https://doi.org/10.1038/nature10353
185 rdf:type schema:CreativeWork
186 sg:pub.10.1038/ncb2848 schema:sameAs https://app.dimensions.ai/details/publication/pub.1053370412
187 https://doi.org/10.1038/ncb2848
188 rdf:type schema:CreativeWork
189 sg:pub.10.1038/nrm3249 schema:sameAs https://app.dimensions.ai/details/publication/pub.1007855841
190 https://doi.org/10.1038/nrm3249
191 rdf:type schema:CreativeWork
192 sg:pub.10.1038/nsmb.2163 schema:sameAs https://app.dimensions.ai/details/publication/pub.1025911101
193 https://doi.org/10.1038/nsmb.2163
194 rdf:type schema:CreativeWork
195 sg:pub.10.1186/2051-5960-1-59 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032216362
196 https://doi.org/10.1186/2051-5960-1-59
197 rdf:type schema:CreativeWork
198 https://doi.org/10.1001/archneurol.2010.52 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008955251
199 rdf:type schema:CreativeWork
200 https://doi.org/10.1016/0092-8674(86)90597-0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1026299955
201 rdf:type schema:CreativeWork
202 https://doi.org/10.1016/j.brainres.2007.06.045 schema:sameAs https://app.dimensions.ai/details/publication/pub.1012507337
203 rdf:type schema:CreativeWork
204 https://doi.org/10.1016/j.cell.2007.10.035 schema:sameAs https://app.dimensions.ai/details/publication/pub.1011978647
205 rdf:type schema:CreativeWork
206 https://doi.org/10.1016/j.cell.2009.03.048 schema:sameAs https://app.dimensions.ai/details/publication/pub.1001834748
207 rdf:type schema:CreativeWork
208 https://doi.org/10.1016/j.cell.2013.05.037 schema:sameAs https://app.dimensions.ai/details/publication/pub.1012347412
209 rdf:type schema:CreativeWork
210 https://doi.org/10.1016/j.febslet.2011.01.045 schema:sameAs https://app.dimensions.ai/details/publication/pub.1025161672
211 rdf:type schema:CreativeWork
212 https://doi.org/10.1016/j.molcel.2009.01.021 schema:sameAs https://app.dimensions.ai/details/publication/pub.1014194959
213 rdf:type schema:CreativeWork
214 https://doi.org/10.1016/j.neulet.2009.11.055 schema:sameAs https://app.dimensions.ai/details/publication/pub.1002574965
215 rdf:type schema:CreativeWork
216 https://doi.org/10.1016/j.neulet.2012.10.003 schema:sameAs https://app.dimensions.ai/details/publication/pub.1017806747
217 rdf:type schema:CreativeWork
218 https://doi.org/10.1016/j.neurobiolaging.2012.02.009 schema:sameAs https://app.dimensions.ai/details/publication/pub.1044071686
219 rdf:type schema:CreativeWork
220 https://doi.org/10.1016/j.neurobiolaging.2014.07.026 schema:sameAs https://app.dimensions.ai/details/publication/pub.1007269184
221 rdf:type schema:CreativeWork
222 https://doi.org/10.1038/emboj.2012.261 schema:sameAs https://app.dimensions.ai/details/publication/pub.1053326953
223 rdf:type schema:CreativeWork
224 https://doi.org/10.1038/embor.2008.238 schema:sameAs https://app.dimensions.ai/details/publication/pub.1043164473
225 rdf:type schema:CreativeWork
226 https://doi.org/10.1074/jbc.m111.333450 schema:sameAs https://app.dimensions.ai/details/publication/pub.1050921545
227 rdf:type schema:CreativeWork
228 https://doi.org/10.1074/jbc.m405988200 schema:sameAs https://app.dimensions.ai/details/publication/pub.1012141205
229 rdf:type schema:CreativeWork
230 https://doi.org/10.1074/jbc.m603337200 schema:sameAs https://app.dimensions.ai/details/publication/pub.1048312989
231 rdf:type schema:CreativeWork
232 https://doi.org/10.1074/jbc.m702824200 schema:sameAs https://app.dimensions.ai/details/publication/pub.1040304237
233 rdf:type schema:CreativeWork
234 https://doi.org/10.1083/jcb.125.2.225 schema:sameAs https://app.dimensions.ai/details/publication/pub.1022398138
235 rdf:type schema:CreativeWork
236 https://doi.org/10.1083/jcb.200803137 schema:sameAs https://app.dimensions.ai/details/publication/pub.1020971115
237 rdf:type schema:CreativeWork
238 https://doi.org/10.1083/jcb.201003122 schema:sameAs https://app.dimensions.ai/details/publication/pub.1046751612
239 rdf:type schema:CreativeWork
240 https://doi.org/10.1083/jcb.201202061 schema:sameAs https://app.dimensions.ai/details/publication/pub.1026456811
241 rdf:type schema:CreativeWork
242 https://doi.org/10.1083/jcb.201302044 schema:sameAs https://app.dimensions.ai/details/publication/pub.1033173546
243 rdf:type schema:CreativeWork
244 https://doi.org/10.1091/mbc.12.11.3690 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029254724
245 rdf:type schema:CreativeWork
246 https://doi.org/10.1093/brain/awt030 schema:sameAs https://app.dimensions.ai/details/publication/pub.1006306003
247 rdf:type schema:CreativeWork
248 https://doi.org/10.1093/nar/gkq837 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008818732
249 rdf:type schema:CreativeWork
250 https://doi.org/10.1097/nen.0b013e3182160690 schema:sameAs https://app.dimensions.ai/details/publication/pub.1027579372
251 rdf:type schema:CreativeWork
252 https://doi.org/10.1111/j.1440-1789.2010.01129.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1033296861
253 rdf:type schema:CreativeWork
254 https://doi.org/10.1111/j.1600-0854.2010.01086.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1001334821
255 rdf:type schema:CreativeWork
256 https://doi.org/10.1111/neup.12058 schema:sameAs https://app.dimensions.ai/details/publication/pub.1016101043
257 rdf:type schema:CreativeWork
258 https://doi.org/10.1126/science.1165942 schema:sameAs https://app.dimensions.ai/details/publication/pub.1030298206
259 rdf:type schema:CreativeWork
260 https://doi.org/10.1126/science.1166066 schema:sameAs https://app.dimensions.ai/details/publication/pub.1044437162
261 rdf:type schema:CreativeWork
262 https://doi.org/10.1136/jmg.36.10.739 schema:sameAs https://app.dimensions.ai/details/publication/pub.1009808839
263 rdf:type schema:CreativeWork
264 https://doi.org/10.1136/jnnp.2009.194399 schema:sameAs https://app.dimensions.ai/details/publication/pub.1044746706
265 rdf:type schema:CreativeWork
266 https://doi.org/10.1155/2012/189794 schema:sameAs https://app.dimensions.ai/details/publication/pub.1042508093
267 rdf:type schema:CreativeWork
268 https://doi.org/10.1242/jcs.03172 schema:sameAs https://app.dimensions.ai/details/publication/pub.1019146455
269 rdf:type schema:CreativeWork
270 https://doi.org/10.14814/phy2.12271 schema:sameAs https://app.dimensions.ai/details/publication/pub.1028569388
271 rdf:type schema:CreativeWork
272 https://doi.org/10.18632/aging.100385 schema:sameAs https://app.dimensions.ai/details/publication/pub.1068670695
273 rdf:type schema:CreativeWork
274 https://doi.org/10.4161/auto.19496 schema:sameAs https://app.dimensions.ai/details/publication/pub.1026175635
275 rdf:type schema:CreativeWork
276 https://doi.org/10.4161/auto.26917 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072289899
277 rdf:type schema:CreativeWork
278 https://doi.org/10.4161/auto.3419 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072290120
279 rdf:type schema:CreativeWork
280 https://doi.org/10.4161/auto.5.5.8566 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072290409
281 rdf:type schema:CreativeWork
282 https://doi.org/10.4161/auto.5524 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072290547
283 rdf:type schema:CreativeWork
284 https://doi.org/10.4161/auto.7.4.14541 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072291013
285 rdf:type schema:CreativeWork
286 https://doi.org/10.4161/auto.8840 schema:sameAs https://app.dimensions.ai/details/publication/pub.1072291173
287 rdf:type schema:CreativeWork
288 https://www.grid.ac/institutes/grid.1004.5 schema:alternateName Macquarie University
289 schema:name Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Macquarie University, North Ryde, New South Wales, Australia
290 rdf:type schema:Organization
291 https://www.grid.ac/institutes/grid.1009.8 schema:alternateName University of Tasmania
292 schema:name Wicking Dementia Research and Education Centre, University of Tasmania, Hobart, Tasmania, Australia
293 rdf:type schema:Organization
294 https://www.grid.ac/institutes/grid.1018.8 schema:alternateName La Trobe University
295 schema:name Department of Biochemistry and Genetics, La Trobe Institute for Molecular Science, La Trobe University, Bundoora, Victoria, Australia
296 rdf:type schema:Organization
 




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


...