Elevation of lung surfactant phosphatidylcholine in mouse models of Sandhoff and of Niemann–Pick A disease View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2004-09

AUTHORS

R. Buccoliero, L. Ginzburg, A. H. Futerman>

ABSTRACT

Sandhoff disease is caused by the defective activity of the lysosomal enzyme beta-hexosaminidase, resulting in accumulation of the glycolipids, GA2 and GM2. Niemann-Pick A/B disease is caused by the defective activity of lysosomal acid sphingomyelinase resulting in sphingomyelin accumulation. Pulmonary complications have been observed in both diseases. We now demonstrate changes in phospholipid levels in pulmonary surfactant in mouse models of these diseases. In the Hexb mouse, a model of Sandhoff disease, lipid phosphate levels were elevated in surfactant from 3- and 4-month-old mice, which was mainly due to elevated levels of phosphatidylcholine. In the ASM mouse, a model of Niemann-Pick A disease, levels of the primary storage material, sphingomyelin, were elevated as expected, and levels of phosphatidylcholine and two other phospholipids were also significantly elevated in pulmonary surfactant and in lung tissue from 5-, 6- and 7-month-old mice. These results suggest that changes in phospholipid levels and composition in lung surfactant might be a general feature of sphingolipid storage diseases, which may be in part responsible for the increased susceptibility of these patients to respiratory infections and lung pathology, often the main reason for the death of these patients. More... »

PAGES

641-648

Journal

TITLE

Journal of Inherited Metabolic Disease

ISSUE

5

VOLUME

27

Author Affiliations

Related Patents

  • Modified Polynucleotides For The Production Of Secreted Proteins
  • Engineered Nucleic Acids Encoding A Modified Erythropoietin And Their Expression
  • Modified Polynucleotides For The Production Of Biologics And Proteins Associated With Human Disease
  • Modified Polynucleotides Encoding Granulysin
  • Modified Polynucleotides For Treating Galactosylceramidase Protein Deficiency
  • Modified Nucleosides, Nucleotides, And Nucleic Acids, And Uses Thereof
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Modified Polynucleotides Encoding Cited4
  • Modified Polynucleotides For The Production Of Nuclear Proteins
  • Modified Polynucleotides Encoding Septin-4
  • Method Of Treating Pulmonary Surfactant Dysfunction Or Respiratory Distress Syndrome By Administering Cyclodextrin Or A Derivative Thereof
  • Compositions And Methods For Reprogramming Eukaryotic Cells
  • Modified Polynucleotides For Treating Protein Deficiency
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Modified Polynucleotides Encoding Apoptosis Inducing Factor 1
  • Delivery And Formulation Of Engineered Nucleic Acids
  • Delivery And Formulation Of Engineered Nucleic Acids
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Modified Polynucleotides For The Production Of Proteins Associated With Human Disease
  • Formulation And Delivery Of Plga Microspheres
  • Compositions And Methods Of Altering Cholesterol Levels
  • Compositions And Methods For Reprogramming Eukaryotic Cells
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Modified Polynucleotides Encoding Copper Metabolism (Murr1) Domain Containing 1
  • Modified Polynucleotides Encoding Basic Helix-Loop-Helix Family Member E41
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Modified Polynucleotides For The Production Of Proteins
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Modified Polynucleotides For The Production Of Oncology-Related Proteins And Peptides
  • Modified Polynucleotides For The Production Of G-Csf
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Rna Containing Modified Nucleosides And Methods Of Use Thereof
  • Terminally Modified Rna
  • Modified Nucleosides, Nucleotides, And Nucleic Acids, And Uses Thereof
  • Engineered Nucleic Acids And Methods Of Use Thereof
  • Modified Polynucleotides For The Production Of Cosmetic Proteins And Peptides
  • Modified Polynucleotides Encoding Siah E3 Ubiquitin Protein Ligase 1
  • Dlin-Kc2-Dma Lipid Nanoparticle Delivery Of Modified Polynucleotides
  • Modified Polynucleotides For The Production Of Secreted Proteins
  • Treatment Of Pulmonary Surfactant Dysfunction Using Cholesterol-Sequestering Cyclodextrins
  • Modified Polynucleotides Encoding Hepatitis A Virus Cellular Receptor 2
  • In Vivo Production Of Proteins
  • Modified Polynucleotides For The Production Of Proteins
  • Modified Nucleosides, Nucleotides, And Nucleic Acids, And Uses Thereof
  • Method For Producing A Protein Of Interest In A Primate
  • Modified Polynucleotides Encoding Aquaporin-5
  • Modified Polynucleotides For The Production Of Proteins Associated With Blood And Lymphatic Disorders
  • Modified Polynucleotides For The Production Of Cytoplasmic And Cytoskeletal Proteins
  • Modified Polynucleotides For The Production Of Cytoplasmic And Cytoskeletal Proteins
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Split Dose Administration
  • Modified Polynucleotides Encoding Aryl Hydrocarbon Receptor Nuclear Translocator
  • Rna Preparations Comprising Purified Modified Rna For Reprogramming Cells
  • Modified Nucleosides, Nucleotides, And Nucleic Acids, And Uses Thereof
  • Modified Polynucleotides For The Production Of Proteins Associated With Blood And Lymphatic Disorders
  • Modified Polynucleotides For The Production Of Biologics And Proteins Associated With Human Disease
  • Formulation And Delivery Of Plga Microspheres
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1023/b:boli.0000042958.22066.6c

    DOI

    http://dx.doi.org/10.1023/b:boli.0000042958.22066.6c

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1006586506

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/15669680


    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/2008/1102", 
            "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
            "name": "Cardiorespiratory Medicine and Haematology", 
            "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"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Animals", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Disease Models, Animal", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Heterozygote", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Humans", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Lipid Metabolism", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Lipids", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Lung", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Mice", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Niemann-Pick Diseases", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Phosphates", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Phosphatidylcholines", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Phospholipids", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Pulmonary Surfactants", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Sandhoff Disease", 
            "type": "DefinedTerm"
          }, 
          {
            "inDefinedTermSet": "https://www.nlm.nih.gov/mesh/", 
            "name": "Time Factors", 
            "type": "DefinedTerm"
          }
        ], 
        "author": [
          {
            "affiliation": {
              "alternateName": "Weizmann Institute of Science", 
              "id": "https://www.grid.ac/institutes/grid.13992.30", 
              "name": [
                "Department of Biological Chemistry, Weizmann Institute of Science, Rehovot, Israel"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Buccoliero", 
            "givenName": "R.", 
            "id": "sg:person.0676143527.86", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0676143527.86"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Weizmann Institute of Science", 
              "id": "https://www.grid.ac/institutes/grid.13992.30", 
              "name": [
                "Department of Biological Chemistry, Weizmann Institute of Science, Rehovot, Israel"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Ginzburg", 
            "givenName": "L.", 
            "id": "sg:person.01357216415.14", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01357216415.14"
            ], 
            "type": "Person"
          }, 
          {
            "affiliation": {
              "alternateName": "Weizmann Institute of Science", 
              "id": "https://www.grid.ac/institutes/grid.13992.30", 
              "name": [
                "Department of Biological Chemistry, Weizmann Institute of Science, Rehovot, Israel", 
                "Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel. E-mail"
              ], 
              "type": "Organization"
            }, 
            "familyName": "Futerman>", 
            "givenName": "A. H.", 
            "id": "sg:person.01004454536.30", 
            "sameAs": [
              "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01004454536.30"
            ], 
            "type": "Person"
          }
        ], 
        "citation": [
          {
            "id": "https://doi.org/10.1164/ajrccm/147.1.218", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1003486985"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1172/jci115523", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1004232257"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1007/bf00180100", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1004659240", 
              "https://doi.org/10.1007/bf00180100"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1080/15513810109168623", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1008702293"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1016/j.bcmd.2004.03.001", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1008972911"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1046/j.1365-2990.2003.00455.x", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1009553567"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1164/rccm.2109105", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1016304241"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1096/fj.02-0149fje", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1019792923"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1053/rmed.2000.0942", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1021599725"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng1095-170", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1021819814", 
              "https://doi.org/10.1038/ng1095-170"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1164/ajrccm.153.1.8542113", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1023508810"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1016/0076-6879(94)30024-0", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1024292363"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1016/0003-2697(76)90527-3", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1025529346"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/ng0795-288", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1027527727", 
              "https://doi.org/10.1038/ng0795-288"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1111/j.1471-4159.2004.02457.x", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1027619870"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1515/cclm.2001.018", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1028904734"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1016/s0344-0338(97)80025-8", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1030676664"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1074/jbc.m302964200", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1032266522"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1164/ajrccm.165.1.2103113", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1032290549"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1159/000196305", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1039236882"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1136/thx.38.11.873", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1040401668"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1136/thx.38.11.873", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1040401668"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1001/archinte.1973.00320070105012", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1042441508"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1034/j.1399-3003.2000.15b22.x", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1042709984"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/labinvest.3780311", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1043756015", 
              "https://doi.org/10.1038/labinvest.3780311"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "sg:pub.10.1038/labinvest.3780311", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1043756015", 
              "https://doi.org/10.1038/labinvest.3780311"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1016/s0076-6879(00)12902-7", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1051416833"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1097/00004728-199611000-00023", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1060189131"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1139/o84-146", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1062894149"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1152/ajplung.00258.2002", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1063195424"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1259/bjr.74.887.741059", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1064567422"
            ], 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://app.dimensions.ai/details/publication/pub.1074694317", 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://app.dimensions.ai/details/publication/pub.1075727656", 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://app.dimensions.ai/details/publication/pub.1075899331", 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://app.dimensions.ai/details/publication/pub.1077411277", 
            "type": "CreativeWork"
          }, 
          {
            "id": "https://doi.org/10.1152/ajplung.1996.270.4.l650", 
            "sameAs": [
              "https://app.dimensions.ai/details/publication/pub.1083003050"
            ], 
            "type": "CreativeWork"
          }
        ], 
        "datePublished": "2004-09", 
        "datePublishedReg": "2004-09-01", 
        "description": "Sandhoff disease is caused by the defective activity of the lysosomal enzyme beta-hexosaminidase, resulting in accumulation of the glycolipids, GA2 and GM2. Niemann-Pick A/B disease is caused by the defective activity of lysosomal acid sphingomyelinase resulting in sphingomyelin accumulation. Pulmonary complications have been observed in both diseases. We now demonstrate changes in phospholipid levels in pulmonary surfactant in mouse models of these diseases. In the Hexb mouse, a model of Sandhoff disease, lipid phosphate levels were elevated in surfactant from 3- and 4-month-old mice, which was mainly due to elevated levels of phosphatidylcholine. In the ASM mouse, a model of Niemann-Pick A disease, levels of the primary storage material, sphingomyelin, were elevated as expected, and levels of phosphatidylcholine and two other phospholipids were also significantly elevated in pulmonary surfactant and in lung tissue from 5-, 6- and 7-month-old mice. These results suggest that changes in phospholipid levels and composition in lung surfactant might be a general feature of sphingolipid storage diseases, which may be in part responsible for the increased susceptibility of these patients to respiratory infections and lung pathology, often the main reason for the death of these patients.", 
        "genre": "research_article", 
        "id": "sg:pub.10.1023/b:boli.0000042958.22066.6c", 
        "inLanguage": [
          "en"
        ], 
        "isAccessibleForFree": false, 
        "isPartOf": [
          {
            "id": "sg:journal.1090550", 
            "issn": [
              "0141-8955", 
              "1573-2665"
            ], 
            "name": "Journal of Inherited Metabolic Disease", 
            "type": "Periodical"
          }, 
          {
            "issueNumber": "5", 
            "type": "PublicationIssue"
          }, 
          {
            "type": "PublicationVolume", 
            "volumeNumber": "27"
          }
        ], 
        "name": "Elevation of lung surfactant phosphatidylcholine in mouse models of Sandhoff and of Niemann\u2013Pick A disease", 
        "pagination": "641-648", 
        "productId": [
          {
            "name": "readcube_id", 
            "type": "PropertyValue", 
            "value": [
              "1f5f36ac3e1e279f6e36d0fb866a9a3e2c79cd43903c05968443a64f16420958"
            ]
          }, 
          {
            "name": "pubmed_id", 
            "type": "PropertyValue", 
            "value": [
              "15669680"
            ]
          }, 
          {
            "name": "nlm_unique_id", 
            "type": "PropertyValue", 
            "value": [
              "7910918"
            ]
          }, 
          {
            "name": "doi", 
            "type": "PropertyValue", 
            "value": [
              "10.1023/b:boli.0000042958.22066.6c"
            ]
          }, 
          {
            "name": "dimensions_id", 
            "type": "PropertyValue", 
            "value": [
              "pub.1006586506"
            ]
          }
        ], 
        "sameAs": [
          "https://doi.org/10.1023/b:boli.0000042958.22066.6c", 
          "https://app.dimensions.ai/details/publication/pub.1006586506"
        ], 
        "sdDataset": "articles", 
        "sdDatePublished": "2019-04-10T15:49", 
        "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_8664_00000503.jsonl", 
        "type": "ScholarlyArticle", 
        "url": "http://link.springer.com/10.1023%2FB%3ABOLI.0000042958.22066.6c"
      }
    ]
     

    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.10.1023/b:boli.0000042958.22066.6c'

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

    curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1023/b:boli.0000042958.22066.6c'

    Turtle is a human-readable linked data format.

    curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1023/b:boli.0000042958.22066.6c'

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

    curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1023/b:boli.0000042958.22066.6c'


     

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

    246 TRIPLES      21 PREDICATES      78 URIs      36 LITERALS      24 BLANK NODES

    Subject Predicate Object
    1 sg:pub.10.1023/b:boli.0000042958.22066.6c schema:about N12ea35dbb2434732850c345b93aa17be
    2 N19e7120b676d47e49da7f60f56354a26
    3 N1c41b3efb37f448891a9a3a9efe5ec56
    4 N287614b68ccb4f1c9d55a8d002f0e6c4
    5 N439f3783d6cb492186802e986ff77af3
    6 N620b88782374448baddba08c79bcb010
    7 Na129f783e3d64b959da692a25527ac42
    8 Naeeb57b2c26f45a99292adaa1e130c0e
    9 Nb7c0ef5093714922907dce3ebf999de8
    10 Nbef016ffe72649fca55bd3f69856d74a
    11 Nd59c4b45ea0a466fbcaf695d5c43188f
    12 Ndea83de453024e3ab88ec51dbf350bdd
    13 Ne92a1852777d4b24b541be6f891a2614
    14 Neee9510ec7614dafae349f8986e94341
    15 Nfd0c36f14dbd4d3abce0e6140a97ffb4
    16 anzsrc-for:11
    17 anzsrc-for:1102
    18 schema:author Na84030205bae4aea9a46a0e706209fef
    19 schema:citation sg:pub.10.1007/bf00180100
    20 sg:pub.10.1038/labinvest.3780311
    21 sg:pub.10.1038/ng0795-288
    22 sg:pub.10.1038/ng1095-170
    23 https://app.dimensions.ai/details/publication/pub.1074694317
    24 https://app.dimensions.ai/details/publication/pub.1075727656
    25 https://app.dimensions.ai/details/publication/pub.1075899331
    26 https://app.dimensions.ai/details/publication/pub.1077411277
    27 https://doi.org/10.1001/archinte.1973.00320070105012
    28 https://doi.org/10.1016/0003-2697(76)90527-3
    29 https://doi.org/10.1016/0076-6879(94)30024-0
    30 https://doi.org/10.1016/j.bcmd.2004.03.001
    31 https://doi.org/10.1016/s0076-6879(00)12902-7
    32 https://doi.org/10.1016/s0344-0338(97)80025-8
    33 https://doi.org/10.1034/j.1399-3003.2000.15b22.x
    34 https://doi.org/10.1046/j.1365-2990.2003.00455.x
    35 https://doi.org/10.1053/rmed.2000.0942
    36 https://doi.org/10.1074/jbc.m302964200
    37 https://doi.org/10.1080/15513810109168623
    38 https://doi.org/10.1096/fj.02-0149fje
    39 https://doi.org/10.1097/00004728-199611000-00023
    40 https://doi.org/10.1111/j.1471-4159.2004.02457.x
    41 https://doi.org/10.1136/thx.38.11.873
    42 https://doi.org/10.1139/o84-146
    43 https://doi.org/10.1152/ajplung.00258.2002
    44 https://doi.org/10.1152/ajplung.1996.270.4.l650
    45 https://doi.org/10.1159/000196305
    46 https://doi.org/10.1164/ajrccm.153.1.8542113
    47 https://doi.org/10.1164/ajrccm.165.1.2103113
    48 https://doi.org/10.1164/ajrccm/147.1.218
    49 https://doi.org/10.1164/rccm.2109105
    50 https://doi.org/10.1172/jci115523
    51 https://doi.org/10.1259/bjr.74.887.741059
    52 https://doi.org/10.1515/cclm.2001.018
    53 schema:datePublished 2004-09
    54 schema:datePublishedReg 2004-09-01
    55 schema:description Sandhoff disease is caused by the defective activity of the lysosomal enzyme beta-hexosaminidase, resulting in accumulation of the glycolipids, GA2 and GM2. Niemann-Pick A/B disease is caused by the defective activity of lysosomal acid sphingomyelinase resulting in sphingomyelin accumulation. Pulmonary complications have been observed in both diseases. We now demonstrate changes in phospholipid levels in pulmonary surfactant in mouse models of these diseases. In the Hexb mouse, a model of Sandhoff disease, lipid phosphate levels were elevated in surfactant from 3- and 4-month-old mice, which was mainly due to elevated levels of phosphatidylcholine. In the ASM mouse, a model of Niemann-Pick A disease, levels of the primary storage material, sphingomyelin, were elevated as expected, and levels of phosphatidylcholine and two other phospholipids were also significantly elevated in pulmonary surfactant and in lung tissue from 5-, 6- and 7-month-old mice. These results suggest that changes in phospholipid levels and composition in lung surfactant might be a general feature of sphingolipid storage diseases, which may be in part responsible for the increased susceptibility of these patients to respiratory infections and lung pathology, often the main reason for the death of these patients.
    56 schema:genre research_article
    57 schema:inLanguage en
    58 schema:isAccessibleForFree false
    59 schema:isPartOf N6875b42cfb924050bc62eb3e4fa61975
    60 Ned994005bdec4234b9e440b61e22a9df
    61 sg:journal.1090550
    62 schema:name Elevation of lung surfactant phosphatidylcholine in mouse models of Sandhoff and of Niemann–Pick A disease
    63 schema:pagination 641-648
    64 schema:productId N72e18e3604a8441f8dbfb2c756ab2a99
    65 N987b437d4c1b4f55b8b0ce385d1eb336
    66 N99ecf1949eec440f9c40c5d93c729de8
    67 Na67d3184e6e743828eed999c0b4f5fe8
    68 Nb7b0ff29bd0b49b896b0f24664081215
    69 schema:sameAs https://app.dimensions.ai/details/publication/pub.1006586506
    70 https://doi.org/10.1023/b:boli.0000042958.22066.6c
    71 schema:sdDatePublished 2019-04-10T15:49
    72 schema:sdLicense https://scigraph.springernature.com/explorer/license/
    73 schema:sdPublisher Naad453b56a4f402a8673974fae9fba44
    74 schema:url http://link.springer.com/10.1023%2FB%3ABOLI.0000042958.22066.6c
    75 sgo:license sg:explorer/license/
    76 sgo:sdDataset articles
    77 rdf:type schema:ScholarlyArticle
    78 N0696da8446564ac097a09ce3da0ba0fa rdf:first sg:person.01004454536.30
    79 rdf:rest rdf:nil
    80 N12ea35dbb2434732850c345b93aa17be schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    81 schema:name Pulmonary Surfactants
    82 rdf:type schema:DefinedTerm
    83 N19e7120b676d47e49da7f60f56354a26 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    84 schema:name Niemann-Pick Diseases
    85 rdf:type schema:DefinedTerm
    86 N1c41b3efb37f448891a9a3a9efe5ec56 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    87 schema:name Sandhoff Disease
    88 rdf:type schema:DefinedTerm
    89 N287614b68ccb4f1c9d55a8d002f0e6c4 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    90 schema:name Animals
    91 rdf:type schema:DefinedTerm
    92 N439f3783d6cb492186802e986ff77af3 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    93 schema:name Lipids
    94 rdf:type schema:DefinedTerm
    95 N620b88782374448baddba08c79bcb010 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    96 schema:name Lung
    97 rdf:type schema:DefinedTerm
    98 N6875b42cfb924050bc62eb3e4fa61975 schema:issueNumber 5
    99 rdf:type schema:PublicationIssue
    100 N72e18e3604a8441f8dbfb2c756ab2a99 schema:name pubmed_id
    101 schema:value 15669680
    102 rdf:type schema:PropertyValue
    103 N987b437d4c1b4f55b8b0ce385d1eb336 schema:name dimensions_id
    104 schema:value pub.1006586506
    105 rdf:type schema:PropertyValue
    106 N99ecf1949eec440f9c40c5d93c729de8 schema:name readcube_id
    107 schema:value 1f5f36ac3e1e279f6e36d0fb866a9a3e2c79cd43903c05968443a64f16420958
    108 rdf:type schema:PropertyValue
    109 Na129f783e3d64b959da692a25527ac42 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    110 schema:name Mice
    111 rdf:type schema:DefinedTerm
    112 Na67d3184e6e743828eed999c0b4f5fe8 schema:name nlm_unique_id
    113 schema:value 7910918
    114 rdf:type schema:PropertyValue
    115 Na84030205bae4aea9a46a0e706209fef rdf:first sg:person.0676143527.86
    116 rdf:rest Nd3b8730fc03c4cd7aee62ef0f47d6fa4
    117 Naad453b56a4f402a8673974fae9fba44 schema:name Springer Nature - SN SciGraph project
    118 rdf:type schema:Organization
    119 Naeeb57b2c26f45a99292adaa1e130c0e schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    120 schema:name Disease Models, Animal
    121 rdf:type schema:DefinedTerm
    122 Nb7b0ff29bd0b49b896b0f24664081215 schema:name doi
    123 schema:value 10.1023/b:boli.0000042958.22066.6c
    124 rdf:type schema:PropertyValue
    125 Nb7c0ef5093714922907dce3ebf999de8 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    126 schema:name Time Factors
    127 rdf:type schema:DefinedTerm
    128 Nbef016ffe72649fca55bd3f69856d74a schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    129 schema:name Lipid Metabolism
    130 rdf:type schema:DefinedTerm
    131 Nd3b8730fc03c4cd7aee62ef0f47d6fa4 rdf:first sg:person.01357216415.14
    132 rdf:rest N0696da8446564ac097a09ce3da0ba0fa
    133 Nd59c4b45ea0a466fbcaf695d5c43188f schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    134 schema:name Phosphatidylcholines
    135 rdf:type schema:DefinedTerm
    136 Ndea83de453024e3ab88ec51dbf350bdd schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    137 schema:name Heterozygote
    138 rdf:type schema:DefinedTerm
    139 Ne92a1852777d4b24b541be6f891a2614 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    140 schema:name Humans
    141 rdf:type schema:DefinedTerm
    142 Ned994005bdec4234b9e440b61e22a9df schema:volumeNumber 27
    143 rdf:type schema:PublicationVolume
    144 Neee9510ec7614dafae349f8986e94341 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    145 schema:name Phosphates
    146 rdf:type schema:DefinedTerm
    147 Nfd0c36f14dbd4d3abce0e6140a97ffb4 schema:inDefinedTermSet https://www.nlm.nih.gov/mesh/
    148 schema:name Phospholipids
    149 rdf:type schema:DefinedTerm
    150 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
    151 schema:name Medical and Health Sciences
    152 rdf:type schema:DefinedTerm
    153 anzsrc-for:1102 schema:inDefinedTermSet anzsrc-for:
    154 schema:name Cardiorespiratory Medicine and Haematology
    155 rdf:type schema:DefinedTerm
    156 sg:journal.1090550 schema:issn 0141-8955
    157 1573-2665
    158 schema:name Journal of Inherited Metabolic Disease
    159 rdf:type schema:Periodical
    160 sg:person.01004454536.30 schema:affiliation https://www.grid.ac/institutes/grid.13992.30
    161 schema:familyName Futerman>
    162 schema:givenName A. H.
    163 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01004454536.30
    164 rdf:type schema:Person
    165 sg:person.01357216415.14 schema:affiliation https://www.grid.ac/institutes/grid.13992.30
    166 schema:familyName Ginzburg
    167 schema:givenName L.
    168 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.01357216415.14
    169 rdf:type schema:Person
    170 sg:person.0676143527.86 schema:affiliation https://www.grid.ac/institutes/grid.13992.30
    171 schema:familyName Buccoliero
    172 schema:givenName R.
    173 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0676143527.86
    174 rdf:type schema:Person
    175 sg:pub.10.1007/bf00180100 schema:sameAs https://app.dimensions.ai/details/publication/pub.1004659240
    176 https://doi.org/10.1007/bf00180100
    177 rdf:type schema:CreativeWork
    178 sg:pub.10.1038/labinvest.3780311 schema:sameAs https://app.dimensions.ai/details/publication/pub.1043756015
    179 https://doi.org/10.1038/labinvest.3780311
    180 rdf:type schema:CreativeWork
    181 sg:pub.10.1038/ng0795-288 schema:sameAs https://app.dimensions.ai/details/publication/pub.1027527727
    182 https://doi.org/10.1038/ng0795-288
    183 rdf:type schema:CreativeWork
    184 sg:pub.10.1038/ng1095-170 schema:sameAs https://app.dimensions.ai/details/publication/pub.1021819814
    185 https://doi.org/10.1038/ng1095-170
    186 rdf:type schema:CreativeWork
    187 https://app.dimensions.ai/details/publication/pub.1074694317 schema:CreativeWork
    188 https://app.dimensions.ai/details/publication/pub.1075727656 schema:CreativeWork
    189 https://app.dimensions.ai/details/publication/pub.1075899331 schema:CreativeWork
    190 https://app.dimensions.ai/details/publication/pub.1077411277 schema:CreativeWork
    191 https://doi.org/10.1001/archinte.1973.00320070105012 schema:sameAs https://app.dimensions.ai/details/publication/pub.1042441508
    192 rdf:type schema:CreativeWork
    193 https://doi.org/10.1016/0003-2697(76)90527-3 schema:sameAs https://app.dimensions.ai/details/publication/pub.1025529346
    194 rdf:type schema:CreativeWork
    195 https://doi.org/10.1016/0076-6879(94)30024-0 schema:sameAs https://app.dimensions.ai/details/publication/pub.1024292363
    196 rdf:type schema:CreativeWork
    197 https://doi.org/10.1016/j.bcmd.2004.03.001 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008972911
    198 rdf:type schema:CreativeWork
    199 https://doi.org/10.1016/s0076-6879(00)12902-7 schema:sameAs https://app.dimensions.ai/details/publication/pub.1051416833
    200 rdf:type schema:CreativeWork
    201 https://doi.org/10.1016/s0344-0338(97)80025-8 schema:sameAs https://app.dimensions.ai/details/publication/pub.1030676664
    202 rdf:type schema:CreativeWork
    203 https://doi.org/10.1034/j.1399-3003.2000.15b22.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1042709984
    204 rdf:type schema:CreativeWork
    205 https://doi.org/10.1046/j.1365-2990.2003.00455.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1009553567
    206 rdf:type schema:CreativeWork
    207 https://doi.org/10.1053/rmed.2000.0942 schema:sameAs https://app.dimensions.ai/details/publication/pub.1021599725
    208 rdf:type schema:CreativeWork
    209 https://doi.org/10.1074/jbc.m302964200 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032266522
    210 rdf:type schema:CreativeWork
    211 https://doi.org/10.1080/15513810109168623 schema:sameAs https://app.dimensions.ai/details/publication/pub.1008702293
    212 rdf:type schema:CreativeWork
    213 https://doi.org/10.1096/fj.02-0149fje schema:sameAs https://app.dimensions.ai/details/publication/pub.1019792923
    214 rdf:type schema:CreativeWork
    215 https://doi.org/10.1097/00004728-199611000-00023 schema:sameAs https://app.dimensions.ai/details/publication/pub.1060189131
    216 rdf:type schema:CreativeWork
    217 https://doi.org/10.1111/j.1471-4159.2004.02457.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1027619870
    218 rdf:type schema:CreativeWork
    219 https://doi.org/10.1136/thx.38.11.873 schema:sameAs https://app.dimensions.ai/details/publication/pub.1040401668
    220 rdf:type schema:CreativeWork
    221 https://doi.org/10.1139/o84-146 schema:sameAs https://app.dimensions.ai/details/publication/pub.1062894149
    222 rdf:type schema:CreativeWork
    223 https://doi.org/10.1152/ajplung.00258.2002 schema:sameAs https://app.dimensions.ai/details/publication/pub.1063195424
    224 rdf:type schema:CreativeWork
    225 https://doi.org/10.1152/ajplung.1996.270.4.l650 schema:sameAs https://app.dimensions.ai/details/publication/pub.1083003050
    226 rdf:type schema:CreativeWork
    227 https://doi.org/10.1159/000196305 schema:sameAs https://app.dimensions.ai/details/publication/pub.1039236882
    228 rdf:type schema:CreativeWork
    229 https://doi.org/10.1164/ajrccm.153.1.8542113 schema:sameAs https://app.dimensions.ai/details/publication/pub.1023508810
    230 rdf:type schema:CreativeWork
    231 https://doi.org/10.1164/ajrccm.165.1.2103113 schema:sameAs https://app.dimensions.ai/details/publication/pub.1032290549
    232 rdf:type schema:CreativeWork
    233 https://doi.org/10.1164/ajrccm/147.1.218 schema:sameAs https://app.dimensions.ai/details/publication/pub.1003486985
    234 rdf:type schema:CreativeWork
    235 https://doi.org/10.1164/rccm.2109105 schema:sameAs https://app.dimensions.ai/details/publication/pub.1016304241
    236 rdf:type schema:CreativeWork
    237 https://doi.org/10.1172/jci115523 schema:sameAs https://app.dimensions.ai/details/publication/pub.1004232257
    238 rdf:type schema:CreativeWork
    239 https://doi.org/10.1259/bjr.74.887.741059 schema:sameAs https://app.dimensions.ai/details/publication/pub.1064567422
    240 rdf:type schema:CreativeWork
    241 https://doi.org/10.1515/cclm.2001.018 schema:sameAs https://app.dimensions.ai/details/publication/pub.1028904734
    242 rdf:type schema:CreativeWork
    243 https://www.grid.ac/institutes/grid.13992.30 schema:alternateName Weizmann Institute of Science
    244 schema:name Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel. E-mail
    245 Department of Biological Chemistry, Weizmann Institute of Science, Rehovot, Israel
    246 rdf:type schema:Organization
     




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


    ...