{"id":"https://openalex.org/W1998447342","doi":"https://doi.org/10.1074/jbc.m110641200","title":"Kidney Sulfatides in Mouse Models of Inherited Glycosphingolipid Disorders","display_name":"Kidney Sulfatides in Mouse Models of Inherited Glycosphingolipid Disorders","publication_year":2002,"publication_date":"2002-06-01","ids":{"openalex":"https://openalex.org/W1998447342","doi":"https://doi.org/10.1074/jbc.m110641200","mag":"1998447342","pmid":"https://pubmed.ncbi.nlm.nih.gov/11919180"},"language":"en","primary_location":{"id":"doi:10.1074/jbc.m110641200","doi":"https://doi.org/10.1074/jbc.m110641200","is_oa":true,"landing_page_url":"https://doi.org/10.1074/jbc.m110641200","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":false,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"hybrid","oa_url":"https://doi.org/10.1074/jbc.m110641200","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5076734902","display_name":"Roger Sandhoff","orcid":"https://orcid.org/0000-0003-3794-9458","observed_orcids":["https://orcid.org/0000-0003-3794-9458"]},"institutions":[{"id":"https://openalex.org/I17937529","display_name":"German Cancer Research Center","ror":"https://ror.org/04cdgtt98","country_code":"DE","type":"facility","lineage":["https://openalex.org/I1305996414","https://openalex.org/I17937529"]}],"countries":["DE"],"is_corresponding":true,"raw_author_name":"Roger Sandhoff","raw_affiliation_strings":["Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany. r.sandhoff@dkfz-heidelberg.de","Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany. r.sandhoff@dkfz-heidelberg.de","institution_ids":["https://openalex.org/I17937529"]},{"raw_affiliation_string":"Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany","institution_ids":["https://openalex.org/I17937529"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5014740994","display_name":"Stefan T. Hepbildikler","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I135140700","display_name":"University of Bonn","ror":"https://ror.org/041nas322","country_code":"DE","type":"education","lineage":["https://openalex.org/I135140700"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Stefan T. Hepbildikler","raw_affiliation_strings":["Kekul\u00e9-Institut f\u00fcr Organische Chemie und Biochemie, Universit\u00e4t Bonn, Gerhard-Domagk-Str. 1, 53121 Bonn, Germany","Kekule\u0301-Institut fu\u0308r Organische Chemie und Biochemie, Universita\u0308t Bonn, Gerhard-Domagk-Str. 1, 53121 Bonn, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Kekul\u00e9-Institut f\u00fcr Organische Chemie und Biochemie, Universit\u00e4t Bonn, Gerhard-Domagk-Str. 1, 53121 Bonn, Germany","institution_ids":["https://openalex.org/I135140700"]},{"raw_affiliation_string":"Kekule\u0301-Institut fu\u0308r Organische Chemie und Biochemie, Universita\u0308t Bonn, Gerhard-Domagk-Str. 1, 53121 Bonn, Germany","institution_ids":["https://openalex.org/I135140700"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5043612440","display_name":"Richard Jennemann","orcid":"https://orcid.org/0000-0002-7944-8523","observed_orcids":["https://orcid.org/0000-0002-7944-8523"]},"institutions":[{"id":"https://openalex.org/I17937529","display_name":"German Cancer Research Center","ror":"https://ror.org/04cdgtt98","country_code":"DE","type":"facility","lineage":["https://openalex.org/I1305996414","https://openalex.org/I17937529"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Richard Jennemann","raw_affiliation_strings":["Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany","institution_ids":["https://openalex.org/I17937529"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111842573","display_name":"Rudolf Geyer","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I200763008","display_name":"Justus-Liebig-Universit\u00e4t Gie\u00dfen","ror":"https://ror.org/033eqas34","country_code":"DE","type":"education","lineage":["https://openalex.org/I200763008"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Rudolf Geyer","raw_affiliation_strings":["Biochemisches Institut am Klinikum der Justus-Liebig-Universit\u00e4t Giessen, 35392 Giessen, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Biochemisches Institut am Klinikum der Justus-Liebig-Universit\u00e4t Giessen, 35392 Giessen, Germany","institution_ids":["https://openalex.org/I200763008"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5058696466","display_name":"Volkmar Gieselmann","orcid":"https://orcid.org/0000-0001-9410-4617","observed_orcids":["https://orcid.org/0000-0001-9410-4617"]},"institutions":[{"id":"https://openalex.org/I135140700","display_name":"University of Bonn","ror":"https://ror.org/041nas322","country_code":"DE","type":"education","lineage":["https://openalex.org/I135140700"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Volkmar Gieselmann","raw_affiliation_strings":["Physiologisch Chemisches Institut, Rheinische Friedrich Wilhelms Universit\u00e4t, 53115 Bonn, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Physiologisch Chemisches Institut, Rheinische Friedrich Wilhelms Universit\u00e4t, 53115 Bonn, Germany","institution_ids":["https://openalex.org/I135140700"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5048492327","display_name":"Richard L. Proia","orcid":"https://orcid.org/0000-0003-0456-1270","observed_orcids":["https://orcid.org/0000-0003-0456-1270"]},"institutions":[{"id":"https://openalex.org/I1299303238","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88","country_code":"US","type":"government","lineage":["https://openalex.org/I1299022934","https://openalex.org/I1299303238"]},{"id":"https://openalex.org/I4210090567","display_name":"National Institute of Diabetes and Digestive and Kidney Diseases","ror":"https://ror.org/00adh9b73","country_code":"US","type":"facility","lineage":["https://openalex.org/I1299022934","https://openalex.org/I1299303238","https://openalex.org/I4210090567"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Richard L. Proia","raw_affiliation_strings":["Genetics of Development and Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Genetics of Development and Disease Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892","institution_ids":["https://openalex.org/I1299303238","https://openalex.org/I4210090567"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5017744511","display_name":"Herbert Wiegandt","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I17937529","display_name":"German Cancer Research Center","ror":"https://ror.org/04cdgtt98","country_code":"DE","type":"facility","lineage":["https://openalex.org/I1305996414","https://openalex.org/I17937529"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Herbert Wiegandt","raw_affiliation_strings":["Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany","institution_ids":["https://openalex.org/I17937529"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5110897701","display_name":"Hermann\u2010Josef Gr\u00f6ne","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I17937529","display_name":"German Cancer Research Center","ror":"https://ror.org/04cdgtt98","country_code":"DE","type":"facility","lineage":["https://openalex.org/I1305996414","https://openalex.org/I17937529"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Hermann-Josef Gr\u00f6ne","raw_affiliation_strings":["Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Deutsches Krebsforschungszentrum Heidelberg, Abteilung f\u00fcr Zellul\u00e4re und Molekulare Pathologie, INF 280, 69120 Heidelberg, Germany","institution_ids":["https://openalex.org/I17937529"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":5,"corresponding_author_ids":["https://openalex.org/A5076734902"],"corresponding_institution_ids":["https://openalex.org/I17937529"],"apc_list":{"value":3430,"currency":"USD","value_usd":3430},"apc_paid":{"value":3430,"currency":"USD","value_usd":3430},"fwci":3.1974,"has_fulltext":false,"cited_by_count":66,"citation_normalized_percentile":{"value":0.90964644,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":89,"max":98},"biblio":{"volume":"277","issue":"23","first_page":"20386","last_page":"20398"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.9961000084877014,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.9961000084877014,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11308","display_name":"Sphingolipid Metabolism and Signaling","score":0.0026000000070780516,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.0005000000237487257,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/sulfatide","display_name":"sulfatide","score":0.9869999885559082},{"id":"https://openalex.org/keywords/glycosphingolipid-metabolism","display_name":"glycosphingolipid metabolism","score":0.7649999856948853},{"id":"https://openalex.org/keywords/kidney","display_name":"kidney","score":0.7730000019073486},{"id":"https://openalex.org/keywords/tay-sachs-disease","display_name":"Tay-Sachs disease","score":0.7250000238418579},{"id":"https://openalex.org/keywords/gm2-ganglioside","display_name":"GM2 ganglioside","score":0.7699999809265137},{"id":"https://openalex.org/keywords/arylsulfatase-a","display_name":"arylsulfatase A","score":0.8429999947547913},{"id":"https://openalex.org/keywords/beta-hexosaminidase","display_name":"beta-hexosaminidase","score":0.8690000176429749},{"id":"https://openalex.org/keywords/gd3-synthase","display_name":"GD3 synthase","score":0.8059999942779541},{"id":"https://openalex.org/keywords/galnac-transferase","display_name":"GalNAc transferase","score":0.9430000185966492},{"id":"https://openalex.org/keywords/gm2-activator-protein","display_name":"GM2 activator protein","score":0.9039999842643738},{"id":"https://openalex.org/keywords/ganglioside-degradation","display_name":"ganglioside degradation","score":0.5170000195503235}],"concepts":[{"id":"https://openalex.org/C2776931407","wikidata":"https://www.wikidata.org/wiki/Q418892","display_name":"Glycosphingolipid","level":2,"score":0.8188977837562561},{"id":"https://openalex.org/C2780871851","wikidata":"https://www.wikidata.org/wiki/Q7636184","display_name":"Sulfatase","level":3,"score":0.751029372215271},{"id":"https://openalex.org/C97343561","wikidata":"https://www.wikidata.org/wiki/Q18026873","display_name":"HEXA","level":2,"score":0.7132776975631714},{"id":"https://openalex.org/C2779247111","wikidata":"https://www.wikidata.org/wiki/Q424837","display_name":"Arylsulfatase A","level":3,"score":0.6889172196388245},{"id":"https://openalex.org/C111204789","wikidata":"https://www.wikidata.org/wiki/Q1313153","display_name":"Hexosaminidase","level":3,"score":0.6747375726699829},{"id":"https://openalex.org/C61705674","wikidata":"https://www.wikidata.org/wiki/Q410395","display_name":"Sphingolipid","level":2,"score":0.6679567694664001},{"id":"https://openalex.org/C2778040462","wikidata":"https://www.wikidata.org/wiki/Q4802708","display_name":"Arylsulfatase","level":3,"score":0.6468420028686523},{"id":"https://openalex.org/C2777851122","wikidata":"https://www.wikidata.org/wiki/Q424213","display_name":"Ceramide","level":3,"score":0.6301458477973938},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.6256322264671326},{"id":"https://openalex.org/C2779390119","wikidata":"https://www.wikidata.org/wiki/Q917227","display_name":"Sandhoff disease","level":3,"score":0.5755982398986816},{"id":"https://openalex.org/C207001950","wikidata":"https://www.wikidata.org/wiki/Q141124","display_name":"In vivo","level":2,"score":0.5326293110847473},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.5317655205726624},{"id":"https://openalex.org/C2777687718","wikidata":"https://www.wikidata.org/wiki/Q410539","display_name":"Globotriaosylceramide","level":4,"score":0.5093384981155396},{"id":"https://openalex.org/C88045685","wikidata":"https://www.wikidata.org/wiki/Q174576","display_name":"Activator (genetics)","level":3,"score":0.49249038100242615},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.4830942153930664},{"id":"https://openalex.org/C104292427","wikidata":"https://www.wikidata.org/wiki/Q899781","display_name":"Protein subunit","level":3,"score":0.4197715222835541},{"id":"https://openalex.org/C2776376580","wikidata":"https://www.wikidata.org/wiki/Q407355","display_name":"Transferase","level":3,"score":0.41680338978767395},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.3458693325519562},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.14204999804496765},{"id":"https://openalex.org/C170493617","wikidata":"https://www.wikidata.org/wiki/Q208467","display_name":"Receptor","level":2,"score":0.125360906124115},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.08765536546707153},{"id":"https://openalex.org/C2779969927","wikidata":"https://www.wikidata.org/wiki/Q916766","display_name":"Enzyme replacement therapy","level":3,"score":0.07965606451034546},{"id":"https://openalex.org/C150903083","wikidata":"https://www.wikidata.org/wiki/Q7108","display_name":"Biotechnology","level":1,"score":0.0},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.0},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.0},{"id":"https://openalex.org/C190283241","wikidata":"https://www.wikidata.org/wiki/Q14599311","display_name":"Apoptosis","level":2,"score":0.0}],"mesh":[{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002855","descriptor_name":"Chromatography, Thin Layer","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002855","descriptor_name":"Chromatography, Thin Layer","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002855","descriptor_name":"Chromatography, Thin Layer","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D005732","descriptor_name":"Gangliosides","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D005732","descriptor_name":"Gangliosides","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D005732","descriptor_name":"Gangliosides","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D007668","descriptor_name":"Kidney","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D007668","descriptor_name":"Kidney","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D007668","descriptor_name":"Kidney","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008052","descriptor_name":"Lipid Metabolism, Inborn Errors","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008817","descriptor_name":"Mice, Mutant Strains","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008817","descriptor_name":"Mice, Mutant Strains","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008817","descriptor_name":"Mice, Mutant Strains","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012015","descriptor_name":"Reference Standards","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012015","descriptor_name":"Reference Standards","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012015","descriptor_name":"Reference Standards","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013433","descriptor_name":"Sulfoglycosphingolipids","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D013433","descriptor_name":"Sulfoglycosphingolipids","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D013433","descriptor_name":"Sulfoglycosphingolipids","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D021241","descriptor_name":"Spectrometry, Mass, Electrospray Ionization","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":false},{"descriptor_ui":"D021241","descriptor_name":"Spectrometry, Mass, Electrospray Ionization","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":false},{"descriptor_ui":"D021241","descriptor_name":"Spectrometry, Mass, Electrospray Ionization","qualifier_ui":"Q000379","qualifier_name":"methods","is_major_topic":false},{"descriptor_ui":"D036103","descriptor_name":"Nanotechnology","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D036103","descriptor_name":"Nanotechnology","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D036103","descriptor_name":"Nanotechnology","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1074/jbc.m110641200","doi":"https://doi.org/10.1074/jbc.m110641200","is_oa":true,"landing_page_url":"https://doi.org/10.1074/jbc.m110641200","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":false,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},{"id":"pmid:11919180","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/11919180","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"The Journal of biological chemistry","raw_type":"Journal Article"}],"best_oa_location":{"id":"doi:10.1074/jbc.m110641200","doi":"https://doi.org/10.1074/jbc.m110641200","is_oa":true,"landing_page_url":"https://doi.org/10.1074/jbc.m110641200","pdf_url":null,"source":{"id":"https://openalex.org/S140251998","display_name":"Journal of Biological Chemistry","issn_l":"0021-9258","issn":["0021-9258","1067-8816","1083-351X"],"is_oa":false,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-100","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Biological Chemistry","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","score":0.6240000128746033,"id":"https://metadata.un.org/sdg/3"}],"sustainable_development_goals_aurora":[{"display_name":"Clean water and sanitation","score":0.44999998807907104,"id":"https://metadata.un.org/sdg/6"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":54,"referenced_works":["https://openalex.org/W155010283","https://openalex.org/W1038790992","https://openalex.org/W1483242733","https://openalex.org/W1534329181","https://openalex.org/W1540100099","https://openalex.org/W1556867369","https://openalex.org/W1561447866","https://openalex.org/W1771712095","https://openalex.org/W1811402536","https://openalex.org/W1843978571","https://openalex.org/W1914565079","https://openalex.org/W1918354798","https://openalex.org/W1964598018","https://openalex.org/W1968497385","https://openalex.org/W1978004329","https://openalex.org/W1992431830","https://openalex.org/W1996149510","https://openalex.org/W2004424525","https://openalex.org/W2009746140","https://openalex.org/W2011785740","https://openalex.org/W2012680961","https://openalex.org/W2014276342","https://openalex.org/W2016140784","https://openalex.org/W2019404217","https://openalex.org/W2020740395","https://openalex.org/W2023641192","https://openalex.org/W2026394997","https://openalex.org/W2029997837","https://openalex.org/W2030932379","https://openalex.org/W2031775276","https://openalex.org/W2038274403","https://openalex.org/W2039796269","https://openalex.org/W2042512150","https://openalex.org/W2048701007","https://openalex.org/W2054655880","https://openalex.org/W2054672013","https://openalex.org/W2060766467","https://openalex.org/W2061397088","https://openalex.org/W2077036443","https://openalex.org/W2077727777","https://openalex.org/W2081105968","https://openalex.org/W2089959156","https://openalex.org/W2092190463","https://openalex.org/W2115024349","https://openalex.org/W2124835860","https://openalex.org/W2128578015","https://openalex.org/W2130417330","https://openalex.org/W2143442977","https://openalex.org/W2155105726","https://openalex.org/W2158756338","https://openalex.org/W2165438024","https://openalex.org/W2185275183","https://openalex.org/W3209992856","https://openalex.org/W4233491437"],"related_works":["https://openalex.org/W2128578015","https://openalex.org/W7208213546","https://openalex.org/W1552728681","https://openalex.org/W324475087","https://openalex.org/W12222704","https://openalex.org/W54245640","https://openalex.org/W1916732518","https://openalex.org/W3098123581","https://openalex.org/W286053468","https://openalex.org/W7162135143"],"abstract_inverted_index":{"Sulfatides":[0],"show":[1],"structural,":[2],"and":[3,62,65,72,76,96,131,135,159,249,253,268],"possibly":[4],"physiological":[5],"similarities":[6],"to":[7,141,152,178,263],"gangliosides.":[8],"Kidney":[9],"dysfunction":[10],"might":[11],"be":[12],"correlated":[13],"with":[14,39,206,244],"changes":[15],"in":[16,22,28,36,111,114,229,266,289],"sulfatides,":[17,89],"the":[18,48,55,88,110,127,184,188,231,234,237,242,245],"major":[19],"acidic":[20],"glycosphingolipids":[21],"this":[23,273],"organ.":[24],"To":[25],"elucidate":[26],"their":[27],"vivo":[29,112,230],"metabolic":[30,239],"pathway":[31],"these":[32],"compounds":[33,215],"were":[34,218],"analyzed":[35],"mice":[37,44],"afflicted":[38],"inherited":[40],"glycosphingolipid":[41],"disorders.":[42],"The":[43,209],"under":[45],"study":[46],"lacked":[47],"genes":[49],"encoding":[50],"either":[51],"beta-hexosaminidase":[52,56,60,155],"alpha-subunit":[53],"(Hexa-/-),":[54],"beta-subunit":[57],"(Hexb-/-),":[58],"both":[59,214],"alpha":[61],"beta-subunits":[63],"(Hexa-/-":[64],"Hexb-/-),":[66],"GD3":[67,70],"synthase":[68,71],"(GD3S-/-),":[69],"GalNAc":[73],"transferase":[74],"(GD3S-/-":[75],"GalNAcT-/-),":[77],"GM2":[78,132,207],"activator":[79],"protein":[80],"(Gm2a-/-),":[81],"or":[82],"arylsulfatase":[83],"A":[84,175],"(ASA-/-).":[85],"Quantification":[86],"of":[87,129,203,213,233,247],"I(3)SO(3)(-)-GalCer":[90],"(SM4s),":[91],"II(3)SO(3)(-)-LacCer":[92],"(SM3),":[93],"II(3)SO(3)(-)-Gg(3)Cer":[94],"(SM2a),":[95],"IV(3,)":[97],"II(3)-(SO(3)(-))(2)-Gg(4)Cer":[98],"(SB1a),":[99],"was":[100,198,261],"performed":[101],"by":[102,154,170,251],"nano-electrospray":[103],"tandem":[104],"mass":[105],"spectrometry.":[106],"We":[107],"conclude":[108,275],"for":[109,126],"situation":[113],"mouse":[115,270],"kidneys":[116],"that:":[117],"1)":[118],"a":[119,193,256],"single":[120],"enzyme":[121],"(GalNAc":[122],"transferase)":[123],"is":[124,144,150,176,182],"responsible":[125],"synthesis":[128],"SM2a":[130,149,204],"from":[133,221,286],"SM3":[134,153],"GM3,":[136],"respectively.":[137],"2)":[138],"In":[139,191],"analogy":[140],"GD1a,":[142],"SB1a":[143],"degraded":[145],"via":[146],"SM2a.":[147],"3)":[148],"hydrolyzed":[151],"S":[156,278],"(Hex":[157],"S)":[158],"Hex":[160,164,277],"A,":[161],"but":[162],"not":[163],"B.":[165],"Both":[166],"enzymes":[167],"are":[168],"supported":[169],"GM2-activator":[171],"protein.":[172],"4)":[173],"Arylsulfatase":[174],"required":[177],"degrade":[179],"SB1a.":[180],"It":[181],"probably":[183,219],"sole":[185],"sphingolipid-sulfatase":[186],"cleaving":[187],"galactosyl-3-sulfate":[189],"bond.":[190],"addition,":[192],"human":[194],"Tay-Sachs":[195],"patient's":[196],"liver":[197],"investigated,":[199],"which":[200],"showed":[201],"accumulation":[202],"along":[205],"storage.":[208],"different":[210,222],"ceramide":[211],"compositions":[212],"indicated":[216],"they":[217],"derived":[220],"cell":[223],"types.":[224],"These":[225],"data":[226],"demonstrate":[227],"that":[228,276],"sulfatides":[232],"ganglio-series":[235],"follow":[236],"same":[238],"pathways":[240],"as":[241],"gangliosides":[243],"replacement":[246],"sulfotransferases":[248],"sulfatases":[250],"sialyltransferases":[252],"sialidases.":[254],"Furthermore,":[255],"novel":[257],"neutral":[258,287],"GSL,":[259],"IV(6)GlcNAcbeta-Gb(4)Cer,":[260],"found":[262],"accumulate":[264],"only":[265],"Hexa-/-":[267],"Hexb-/-":[269],"kidneys.":[271],"From":[272],"we":[274],"also":[279],"efficiently":[280],"cleaves":[281],"terminal":[282],"beta1-6-linked":[283],"HexNAc":[284],"residues":[285],"GSLs":[288],"vivo.":[290]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":3},{"year":2023,"cited_by_count":1},{"year":2022,"cited_by_count":1},{"year":2019,"cited_by_count":5},{"year":2018,"cited_by_count":4},{"year":2017,"cited_by_count":1},{"year":2016,"cited_by_count":1},{"year":2014,"cited_by_count":4},{"year":2013,"cited_by_count":2},{"year":2012,"cited_by_count":5}],"updated_date":"2026-10-12T09:40:04.860316","created_date":"2016-06-24T00:00:00"}