{"doi":"10.1007/s11064-010-0380-3","title":"Secondary Alterations of Sphingolipid Metabolism in Lysosomal Storage Diseases","abstract":null,"journal":"Neurochemical Research","year":2011,"id":616468,"datarank":0.5375278407684165,"base_score":3.58351893845611,"endowment":3.58351893845611,"self_citation_contribution":0.5375278407684165,"citation_network_contribution":0.0,"self_endowment_contribution":0.5375278407684165,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":35,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1589414,"name":"Simona Prioni","orcid":null,"position":1,"is_corresponding":false},{"id":1589416,"name":"Elena Chiricozzi","orcid":null,"position":2,"is_corresponding":false},{"id":330858,"name":"Edward H. 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Moreover, we present a detailed analysis of the lipid composition of different central nervous system and extraneural tissues from the acid sphingomyelinase-deficient mouse, the animal model for Niemann-Pick disease type A, characterized by the accumulation of sphingomyelin. Our data show an unexpected, tissue specific selection of the accumulated molecular species of sphingomyelin, and an accumulation of GM3 and GM2 gangliosides in both neural and extraneural tissues, that cannot be solely explained by the lack of acid sphingomyelinase.","is_dataset_classified":null,"base_score":3.58351893845611,"endowment":3.58351893845611,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"21207141","pmcid":null,"openalex_id":"https://openalex.org/W1998819220","authors":[],"funders":[{"funder_name":"National Institutes of Health","grant_id":"5R01DK054830-15","title":"Acid Ceramidase, Ceramide and Farber Disease"},{"funder_name":"National Institutes of Health","grant_id":"1S15HD028750-01","title":"SMALL INSTRUMENTATION GRANT"}],"total_grants":2,"fwci":1.7667,"citation_percentile":0.83149979,"influential_citations":0,"citation_trend":[{"year":2012,"count":3},{"year":2013,"count":3},{"year":2014,"count":4},{"year":2016,"count":1},{"year":2017,"count":2},{"year":2018,"count":5},{"year":2019,"count":4},{"year":2020,"count":5},{"year":2021,"count":2},{"year":2022,"count":2},{"year":2023,"count":1},{"year":2024,"count":1},{"year":2025,"count":1}],"oa_status":"closed","license":"Springer TDM","oa_locations":[{"url":"http://link.springer.com/content/pdf/10.1007/s11064-010-0380-3.pdf","host_type":"publisher"},{"url":"http://link.springer.com/article/10.1007/s11064-010-0380-3/fulltext.html","host_type":"publisher"},{"url":"http://link.springer.com/content/pdf/10.1007/s11064-010-0380-3","host_type":"publisher"},{"url":"https://doi.org/10.1007/s11064-010-0380-3","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/21207141","host_type":"repository"},{"url":"https://dx.doi.org/10.1007/s11064-010-0380-3","host_type":""},{"url":"https://hdl.handle.net/2434/173844","host_type":""},{"url":"https://hdl.handle.net/2434/171013","host_type":""}],"fields_of_study":["Lysosomal Storage Disorders Research","Sphingolipid Metabolism and Signaling","Cellular transport and secretion","0301 basic medicine","0303 health sciences","03 medical and health sciences","Animals","Gangliosides","Glycosyltransferases","Lipid Metabolism","Lysosomal Storage Diseases","Nervous System","Neurodegenerative Diseases","Niemann-Pick Diseases","Sphingolipids","Tissue Distribution"],"mesh_terms":["Animals","Gangliosides","Nervous System","Niemann-Pick Diseases","Sphingolipids","Tissue Distribution","Lysosomal Storage Diseases","Glycosyltransferases","Neurodegenerative Diseases","Lipid Metabolism"],"keywords":["Sphingolipid","Metabolism","Neurochemistry","Biology","Neurology","Neuroscience","Cell biology","Biochemistry","Niemann-Pick Diseases","Sphingolipids","Gangliosides; Glycosphingolipids; Niemann-Pick disease; Sphingolipidosis; Sphingolipids; Sphingomyelin","Glycosyltransferases","Neurodegenerative Diseases","Lipid Metabolism","sphingolipids; sphingomyelin; glycosphingolipids; gangliosides; sphingolipid storage diseases; Niemann-Pick","Nervous System","Lysosomal Storage Diseases","Gangliosides","Animals","Tissue Distribution"],"sdg_mappings":[{"sdg_number":3,"sdg_label":"3. 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