{"id":"https://openalex.org/W2003370301","doi":"https://doi.org/10.1371/journal.pone.0064647","title":"Central Nervous System Pathology Progresses Independently of KC and CXCR2 in Globoid-Cell Leukodystrophy","display_name":"Central Nervous System Pathology Progresses Independently of KC and CXCR2 in Globoid-Cell Leukodystrophy","publication_year":2013,"publication_date":"2013-06-03","ids":{"openalex":"https://openalex.org/W2003370301","doi":"https://doi.org/10.1371/journal.pone.0064647","mag":"2003370301","pmid":"https://pubmed.ncbi.nlm.nih.gov/23755134","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/3670857"},"language":"en","primary_location":{"id":"doi:10.1371/journal.pone.0064647","doi":"https://doi.org/10.1371/journal.pone.0064647","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0064647","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0064647&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0064647&type=printable","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5017595528","display_name":"Adarsh S. Reddy","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Adarsh S. Reddy","raw_affiliation_strings":["Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5031732446","display_name":"Jigisha R. Patel","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Jigisha R. Patel","raw_affiliation_strings":["Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, United States of America"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5082333244","display_name":"Carole A. Vogler","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I47838141","display_name":"Saint Louis University","ror":"https://ror.org/01p7jjy08","country_code":"US","type":"education","lineage":["https://openalex.org/I47838141"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Carole Vogler","raw_affiliation_strings":["Department of Pathology, St. Louis University School of Medicine, St. Louis, Missouri, United States of America"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, St. Louis University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I47838141"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5046722897","display_name":"Robyn S. Klein","orcid":"https://orcid.org/0000-0003-0281-1352","observed_orcids":["https://orcid.org/0000-0003-0281-1352"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Robyn S. Klein","raw_affiliation_strings":["Department of Anatomy and Neurobiology, Washington University School of Medicine, St. Louis, Missouri, United States of America","Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America","Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, United States of America"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Anatomy and Neurobiology, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]},{"raw_affiliation_string":"Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]},{"raw_affiliation_string":"Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5049345275","display_name":"Mark S. Sands","orcid":"https://orcid.org/0000-0002-5559-0832","observed_orcids":["https://orcid.org/0000-0002-5559-0832"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Mark S. Sands","raw_affiliation_strings":["Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri, United States of America","institution_ids":["https://openalex.org/I204465549"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5017595528","https://openalex.org/A5031732446","https://openalex.org/A5046722897","https://openalex.org/A5049345275","https://openalex.org/A5082333244"],"corresponding_institution_ids":["https://openalex.org/I204465549","https://openalex.org/I47838141"],"apc_list":{"value":2382,"currency":"USD","value_usd":2382},"apc_paid":{"value":2382,"currency":"USD","value_usd":2382},"fwci":1.035,"has_fulltext":true,"cited_by_count":9,"citation_normalized_percentile":{"value":0.75350445,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":97},"biblio":{"volume":"8","issue":"6","first_page":"e64647","last_page":"e64647"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology (medical)"},"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":1.0,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology (medical)"},"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/T11027","display_name":"Metabolism and Genetic Disorders","score":0.0,"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"}},{"id":"https://openalex.org/T12604","display_name":"Glycogen Storage Diseases and Myoclonus","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/krabbe-disease","display_name":"Krabbe disease","score":0.9879999756813049},{"id":"https://openalex.org/keywords/galactosylceramidase-deficiency","display_name":"galactosylceramidase deficiency","score":0.8709999918937683},{"id":"https://openalex.org/keywords/kc-chemokine","display_name":"KC chemokine","score":0.6439999938011169},{"id":"https://openalex.org/keywords/demyelination","display_name":"demyelination","score":0.9110000133514404},{"id":"https://openalex.org/keywords/neuroinflammation","display_name":"neuroinflammation","score":0.8489999771118164},{"id":"https://openalex.org/keywords/macrophage-infiltration","display_name":"macrophage infiltration","score":0.8299999833106995},{"id":"https://openalex.org/keywords/oligodendrocyte-death","display_name":"oligodendrocyte death","score":0.9070000052452087},{"id":"https://openalex.org/keywords/bone-marrow-transplantation","display_name":"bone marrow transplantation","score":0.7820000052452087},{"id":"https://openalex.org/keywords/gene-therapy","display_name":"gene therapy","score":0.9610000252723694},{"id":"https://openalex.org/keywords/cytokine-redundancy","display_name":"cytokine redundancy","score":0.5740000009536743},{"id":"https://openalex.org/keywords/pathogenesis","display_name":"pathogenesis","score":0.5350000262260437}],"concepts":[{"id":"https://openalex.org/C2776100374","wikidata":"https://www.wikidata.org/wiki/Q1821559","display_name":"Leukodystrophy","level":3,"score":0.8854589462280273},{"id":"https://openalex.org/C2777585455","wikidata":"https://www.wikidata.org/wiki/Q511372","display_name":"Krabbe disease","level":4,"score":0.6418872475624084},{"id":"https://openalex.org/C2779830541","wikidata":"https://www.wikidata.org/wiki/Q1622829","display_name":"Microglia","level":3,"score":0.593445897102356},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.5606861114501953},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.5306260585784912},{"id":"https://openalex.org/C2778609137","wikidata":"https://www.wikidata.org/wiki/Q105790271","display_name":"Myelin","level":3,"score":0.5248234868049622},{"id":"https://openalex.org/C87753298","wikidata":"https://www.wikidata.org/wiki/Q17157137","display_name":"Neuroinflammation","level":3,"score":0.5211141705513},{"id":"https://openalex.org/C2780942790","wikidata":"https://www.wikidata.org/wiki/Q372016","display_name":"Pathogenesis","level":2,"score":0.4751049280166626},{"id":"https://openalex.org/C2776985911","wikidata":"https://www.wikidata.org/wiki/Q916698","display_name":"Oligodendrocyte","level":4,"score":0.46940648555755615},{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.4650835394859314},{"id":"https://openalex.org/C2778583010","wikidata":"https://www.wikidata.org/wiki/Q3237081","display_name":"Leukoencephalopathy","level":3,"score":0.46366432309150696},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.4452715814113617},{"id":"https://openalex.org/C529278444","wikidata":"https://www.wikidata.org/wiki/Q47273","display_name":"Central nervous system","level":2,"score":0.3090088665485382},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.30141890048980713},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.2951616644859314},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.17606550455093384},{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.17427077889442444},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.13478636741638184}],"mesh":[{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001853","descriptor_name":"Bone Marrow","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001853","descriptor_name":"Bone Marrow","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D002490","descriptor_name":"Central Nervous System","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D002490","descriptor_name":"Central Nervous System","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005434","descriptor_name":"Flow Cytometry","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005698","descriptor_name":"Galactosylceramidase","qualifier_ui":"Q000172","qualifier_name":"deficiency","is_major_topic":false},{"descriptor_ui":"D005698","descriptor_name":"Galactosylceramidase","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D007249","descriptor_name":"Inflammation","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D007965","descriptor_name":"Leukodystrophy, Globoid Cell","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D007965","descriptor_name":"Leukodystrophy, Globoid Cell","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D008136","descriptor_name":"Longevity","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008159","descriptor_name":"Lumbar Vertebrae","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008159","descriptor_name":"Lumbar Vertebrae","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D008810","descriptor_name":"Mice, Inbred C57BL","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009836","descriptor_name":"Oligodendroglia","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D009836","descriptor_name":"Oligodendroglia","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D018450","descriptor_name":"Disease Progression","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D023063","descriptor_name":"Receptors, Interleukin-8B","qualifier_ui":"Q000172","qualifier_name":"deficiency","is_major_topic":false},{"descriptor_ui":"D023063","descriptor_name":"Receptors, Interleukin-8B","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D049109","descriptor_name":"Cell Proliferation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D054360","descriptor_name":"Chemokine CXCL1","qualifier_ui":"Q000172","qualifier_name":"deficiency","is_major_topic":false},{"descriptor_ui":"D054360","descriptor_name":"Chemokine CXCL1","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true}],"locations_count":7,"locations":[{"id":"doi:10.1371/journal.pone.0064647","doi":"https://doi.org/10.1371/journal.pone.0064647","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0064647","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0064647&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},{"id":"pmh:oai:europepmc.org:2674074","doi":null,"is_oa":true,"landing_page_url":"http://europepmc.org/articles/PMC3670857","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"","raw_type":"Text"},{"id":"pmh:oai:pubmedcentral.nih.gov:3670857","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3670857","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"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":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS One","raw_type":"Text"},{"id":"pmid:23755134","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/23755134","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":"PloS one","raw_type":"Journal Article"},{"id":"pmh:oai:digitalcommons.wustl.edu:open_access_pubs-2577","doi":null,"is_oa":true,"landing_page_url":"https://digitalcommons.wustl.edu/open_access_pubs/1575","pdf_url":"https://digitalcommons.wustl.edu/open_access_pubs/1575","source":{"id":"https://openalex.org/S4306400764","display_name":"Digital Commons@Becker (Washington University School of Medicine)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I204465549","host_organization_name":"Washington University in St. Louis","host_organization_lineage":["https://openalex.org/I204465549"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Open Access Publications","raw_type":"text"},{"id":"pmh:oai:CiteSeerX.psu:10.1.1.788.1442","doi":null,"is_oa":false,"landing_page_url":"http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.788.1442","pdf_url":null,"source":null,"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"ftp://ftp.ncbi.nlm.nih.gov/pub/pmc/ef/69/pone.0064647.PMC3670857.pdf","raw_type":"text"},{"id":"pmh:oai:doaj.org/article:b6d54ba5f817454e85535cf206aefb9f","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/b6d54ba5f817454e85535cf206aefb9f","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"PLoS ONE, Vol 8, Iss 6, p e64647 (2014)","raw_type":"article"}],"best_oa_location":{"id":"doi:10.1371/journal.pone.0064647","doi":"https://doi.org/10.1371/journal.pone.0064647","is_oa":true,"landing_page_url":"https://doi.org/10.1371/journal.pone.0064647","pdf_url":"https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0064647&type=printable","source":{"id":"https://openalex.org/S202381698","display_name":"PLoS ONE","issn_l":"1932-6203","issn":["1932-6203"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","doaj","dongbi-c","doyens","erih-plus","jufo-1","ki-jl-1","medline","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315706","host_organization_name":"Public Library of Science","host_organization_lineage":["https://openalex.org/P4310315706"],"host_organization_lineage_names":["Public Library of Science"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"PLoS ONE","raw_type":"journal-article"},"sustainable_development_goals":[{"score":0.9537000060081482,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[{"id":"https://openalex.org/G4700852728","display_name":null,"funder_award_id":"R01 NS","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"},{"id":"https://openalex.org/G5037538125","display_name":"NOVEL THERAPIES FOR GLOBOID-CELL LEUKODYSTROPHY","funder_award_id":"R01NS084861","funder_id":"https://openalex.org/F4320337359","funder_display_name":"National Institute of Neurological Disorders and Stroke"},{"id":"https://openalex.org/G5441180306","display_name":"Neuroprotection and repair during West Nile Virus Encephalitis","funder_award_id":"R01NS052632","funder_id":"https://openalex.org/F4320337359","funder_display_name":"National Institute of Neurological Disorders and Stroke"},{"id":"https://openalex.org/G5520316217","display_name":null,"funder_award_id":"NIH-R01","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"},{"id":"https://openalex.org/G6068191474","display_name":null,"funder_award_id":"NIH R01 NS 043205","funder_id":"https://openalex.org/F4320337359","funder_display_name":"National Institute of Neurological Disorders and Stroke"},{"id":"https://openalex.org/G6368000310","display_name":"Novel Therapies for Globoid-Cell Leukodystrophy","funder_award_id":"R01HD055461","funder_id":"https://openalex.org/F4320337611","funder_display_name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development"},{"id":"https://openalex.org/G6624740349","display_name":"PATHOPHYSIOLOGY AND NOVEL THERAPIES FOR BATTEN'S DISEASE","funder_award_id":"R01NS043205","funder_id":"https://openalex.org/F4320337359","funder_display_name":"National Institute of Neurological Disorders and Stroke"},{"id":"https://openalex.org/G9000275562","display_name":null,"funder_award_id":"NS 043205","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"}],"funders":[{"id":"https://openalex.org/F4320306161","display_name":"National Multiple Sclerosis Society","ror":"https://ror.org/03019wv17"},{"id":"https://openalex.org/F4320307600","display_name":"Hunter's Hope Foundation","ror":"https://ror.org/00m4t9795"},{"id":"https://openalex.org/F4320314494","display_name":"Hope Foundation","ror":"https://ror.org/05wf1w785"},{"id":"https://openalex.org/F4320332161","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88"},{"id":"https://openalex.org/F4320337359","display_name":"National Institute of Neurological Disorders and Stroke","ror":"https://ror.org/01s5ya894"},{"id":"https://openalex.org/F4320337611","display_name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","ror":"https://ror.org/04byxyr05"}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W2003370301.pdf","grobid_xml":"https://content.openalex.org/works/W2003370301.grobid-xml"},"referenced_works_count":43,"referenced_works":["https://openalex.org/W1481493576","https://openalex.org/W1543813190","https://openalex.org/W1574929990","https://openalex.org/W1765057352","https://openalex.org/W1791569952","https://openalex.org/W1961326234","https://openalex.org/W1965002999","https://openalex.org/W1971989809","https://openalex.org/W1973986115","https://openalex.org/W1977529564","https://openalex.org/W1988438945","https://openalex.org/W1993296615","https://openalex.org/W2000156766","https://openalex.org/W2017132882","https://openalex.org/W2025691049","https://openalex.org/W2025793780","https://openalex.org/W2026320463","https://openalex.org/W2029907954","https://openalex.org/W2035574918","https://openalex.org/W2047286951","https://openalex.org/W2048721791","https://openalex.org/W2049644653","https://openalex.org/W2050707975","https://openalex.org/W2055247780","https://openalex.org/W2058462779","https://openalex.org/W2062133707","https://openalex.org/W2066284508","https://openalex.org/W2079721567","https://openalex.org/W2083183631","https://openalex.org/W2083218881","https://openalex.org/W2089627637","https://openalex.org/W2102935355","https://openalex.org/W2121488001","https://openalex.org/W2126939740","https://openalex.org/W2138659609","https://openalex.org/W2139485237","https://openalex.org/W2147696659","https://openalex.org/W2152381333","https://openalex.org/W2161475784","https://openalex.org/W2167347313","https://openalex.org/W2338260536","https://openalex.org/W2398350456","https://openalex.org/W2764428680"],"related_works":["https://openalex.org/W7095082569","https://openalex.org/W1604609895","https://openalex.org/W4396830310","https://openalex.org/W2026320463","https://openalex.org/W3005368131","https://openalex.org/W1980671800","https://openalex.org/W2020008627","https://openalex.org/W2513045515","https://openalex.org/W359802628","https://openalex.org/W7018059352"],"abstract_inverted_index":{"Globoid-cell":[0],"Leukodystrophy":[1],"(GLD;":[2],"Krabbe's":[3],"disease)":[4],"is":[5,94,124,131,140,179,190],"a":[6,14,40,109,132],"rapidly":[7,41],"progressing":[8],"inherited":[9],"demyelinating":[10],"disease":[11,44,154,178,215],"caused":[12],"by":[13],"deficiency":[15,68],"of":[16,23,29,54,69,80,100,122,159,171,176,204,213,222,229,239],"the":[17,32,59,78,84,98,127,150,153,157,169,174,177,211,214,227,237],"lysosomal":[18],"enzyme":[19,61],"Galactosylceramidase":[20],"(GALC).":[21],"Deficiency":[22],"GALC":[24,70],"leads":[25,38,71],"to":[26,39,72,168],"altered":[27,182],"catabolism":[28],"galactosylceramide":[30],"and":[31,49,62,77,104,130,137,146,156,233],"cytotoxic":[33],"lipid,":[34],"galactosylsphingosine":[35],"(psychosine).":[36],"This":[37,224],"progressive":[42],"fatal":[43],"with":[45,117],"spasticity,":[46],"cognitive":[47],"disability":[48],"seizures.":[50],"The":[51,67,120],"murine":[52],"model":[53],"GLD":[55],"(Twitcher;":[56],"GALC-/-)":[57],"lacks":[58],"same":[60],"has":[63],"similar":[64],"clinical":[65],"features.":[66],"oligodendrocyte":[73,144],"death,":[74],"profound":[75],"neuroinflammation,":[76],"influx":[79],"activated":[81],"macrophages":[82,136],"into":[83],"CNS.":[85],"We":[86],"showed":[87],"previously":[88],"that":[89,163],"keratinocyte":[90],"chemoattractant":[91,134],"factor":[92],"(KC)":[93],"highly":[95],"elevated":[96],"in":[97,143,183,194,199,219,236],"CNS":[99],"untreated":[101],"Twitcher":[102,187],"mice":[103],"significantly":[105,181],"decreases":[106],"after":[107],"receiving":[108],"relatively":[110],"effective":[111],"therapy":[112],"(bone":[113],"marrow":[114,207],"transplantation":[115,203],"combined":[116],"gene":[118],"therapy).":[119],"action":[121],"KC":[123,139,164],"mediated":[125],"through":[126],"CXCR2":[128,166],"receptor":[129],"potent":[133],"for":[135],"microglia.":[138],"also":[141,191],"involved":[142],"migration":[145],"proliferation.":[147],"Based":[148],"on":[149],"commonalities":[151],"between":[152],"presentation":[155],"functions":[158],"KC,":[160],"we":[161],"hypothesized":[162],"and/or":[165],"contribute":[167],"pathogenesis":[170,238],"GLD.":[172,240],"Interestingly,":[173],"course":[175],"not":[180,209],"KC-":[184],"or":[185,196],"CXCR2-deficient":[186,205],"mice.":[188,201],"There":[189],"no":[192],"alteration":[193],"inflammation":[195],"demyelination":[197],"patterns":[198],"these":[200],"Furthermore,":[202],"bone":[206],"does":[208,218],"alter":[210],"progression":[212],"as":[216],"it":[217],"other":[220],"models":[221],"demyelination.":[223],"study":[225],"highlights":[226],"role":[228],"multiple":[230],"redundant":[231],"cytokines":[232],"growth":[234],"factors":[235]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":1},{"year":2020,"cited_by_count":2},{"year":2016,"cited_by_count":3},{"year":2015,"cited_by_count":1},{"year":2014,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
