{"id":"https://openalex.org/W2888316632","doi":"https://doi.org/10.1111/jnc.14577","title":"Fundamentals of CNS energy metabolism and alterations in lysosomal storage diseases","display_name":"Fundamentals of CNS energy metabolism and alterations in lysosomal storage diseases","publication_year":2018,"publication_date":"2018-08-25","ids":{"openalex":"https://openalex.org/W2888316632","doi":"https://doi.org/10.1111/jnc.14577","mag":"2888316632","pmid":"https://pubmed.ncbi.nlm.nih.gov/30144055"},"language":"en","primary_location":{"id":"doi:10.1111/jnc.14577","doi":"https://doi.org/10.1111/jnc.14577","is_oa":false,"landing_page_url":"https://doi.org/10.1111/jnc.14577","pdf_url":null,"source":{"id":"https://openalex.org/S135837084","display_name":"Journal of Neurochemistry","issn_l":"0022-3042","issn":["0022-3042","1471-4159"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-d","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Neurochemistry","raw_type":"journal-article"},"volume":null,"type":"review","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5031666850","display_name":"Mary C. McKenna","orcid":"https://orcid.org/0000-0002-5236-3853","observed_orcids":["https://orcid.org/0000-0002-5236-3853"]},"institutions":[{"id":"https://openalex.org/I126744593","display_name":"University of Maryland, Baltimore","ror":"https://ror.org/04rq5mt64","country_code":"US","type":"education","lineage":["https://openalex.org/I126744593"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Mary C. McKenna","raw_affiliation_strings":["Department of Pediatrics University of Maryland School of Medicine Baltimore Maryland USA","Program in Neuroscience University of Maryland School of Medicine Baltimore Maryland USA","Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, USA","Program in Neuroscience, University of Maryland School of Medicine, Baltimore, Maryland, USA"],"raw_orcid":"https://orcid.org/0000-0002-5236-3853","affiliations":[{"raw_affiliation_string":"Department of Pediatrics University of Maryland School of Medicine Baltimore Maryland USA","institution_ids":["https://openalex.org/I126744593"]},{"raw_affiliation_string":"Program in Neuroscience University of Maryland School of Medicine Baltimore Maryland USA","institution_ids":["https://openalex.org/I126744593"]},{"raw_affiliation_string":"Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, USA","institution_ids":["https://openalex.org/I126744593"]},{"raw_affiliation_string":"Program in Neuroscience, University of Maryland School of Medicine, Baltimore, Maryland, USA","institution_ids":["https://openalex.org/I126744593"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5063336320","display_name":"Patr\u00edcia Fernanda Schuck","orcid":"https://orcid.org/0000-0003-3148-4952","observed_orcids":["https://orcid.org/0000-0003-3148-4952"]},"institutions":[{"id":"https://openalex.org/I45643870","display_name":"Pontif\u00edcia Universidade Cat\u00f3lica do Rio Grande do Sul","ror":"https://ror.org/025vmq686","country_code":"BR","type":"education","lineage":["https://openalex.org/I45643870"]}],"countries":["BR"],"is_corresponding":false,"raw_author_name":"Patricia F. Schuck","raw_affiliation_strings":["School of Sciences Pontifical Catholic University of Rio Grande do Sul Porto Alegre Rio Grande do Sul Brazil","School of Sciences, Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil"],"raw_orcid":"https://orcid.org/0000-0003-3148-4952","affiliations":[{"raw_affiliation_string":"School of Sciences Pontifical Catholic University of Rio Grande do Sul Porto Alegre Rio Grande do Sul Brazil","institution_ids":["https://openalex.org/I45643870"]},{"raw_affiliation_string":"School of Sciences, Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil","institution_ids":["https://openalex.org/I45643870"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5021019594","display_name":"Gustavo C. Ferreira","orcid":"https://orcid.org/0000-0002-2892-8842","observed_orcids":["https://orcid.org/0000-0002-2892-8842"]},"institutions":[{"id":"https://openalex.org/I122140584","display_name":"Universidade Federal do Rio de Janeiro","ror":"https://ror.org/03490as77","country_code":"BR","type":"education","lineage":["https://openalex.org/I122140584"]},{"id":"https://openalex.org/I126744593","display_name":"University of Maryland, Baltimore","ror":"https://ror.org/04rq5mt64","country_code":"US","type":"education","lineage":["https://openalex.org/I126744593"]}],"countries":["BR","US"],"is_corresponding":false,"raw_author_name":"Gustavo C. Ferreira","raw_affiliation_strings":["Department of Pediatrics University of Maryland School of Medicine Baltimore Maryland USA","Institute of Medical Biochemistry Leopoldo de Meis Federal University of Rio de Janeiro Rio de Janeiro  Rio Grande do Sul Brazil","Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, USA","Institute of Medical Biochemistry Leopoldo de Meis, Federal University of Rio de Janeiro, Rio de Janeiro, Rio Grande do Sul, Brazil"],"raw_orcid":"https://orcid.org/0000-0002-2892-8842","affiliations":[{"raw_affiliation_string":"Department of Pediatrics University of Maryland School of Medicine Baltimore Maryland USA","institution_ids":["https://openalex.org/I126744593"]},{"raw_affiliation_string":"Institute of Medical Biochemistry Leopoldo de Meis Federal University of Rio de Janeiro Rio de Janeiro  Rio Grande do Sul Brazil","institution_ids":["https://openalex.org/I122140584"]},{"raw_affiliation_string":"Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, USA","institution_ids":["https://openalex.org/I126744593"]},{"raw_affiliation_string":"Institute of Medical Biochemistry Leopoldo de Meis, Federal University of Rio de Janeiro, Rio de Janeiro, Rio Grande do Sul, Brazil","institution_ids":["https://openalex.org/I122140584"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5031666850"],"corresponding_institution_ids":["https://openalex.org/I126744593"],"apc_list":{"value":5330,"currency":"USD","value_usd":5330},"apc_paid":null,"fwci":0.3945,"has_fulltext":false,"cited_by_count":29,"citation_normalized_percentile":{"value":0.37754225,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":94,"max":99},"biblio":{"volume":"148","issue":"5","first_page":"590","last_page":"599"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.7602999806404114,"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":0.7602999806404114,"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/T12604","display_name":"Glycogen Storage Diseases and Myoclonus","score":0.1784999966621399,"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"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.01850000023841858,"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/brain-energy-metabolism","display_name":"brain energy metabolism","score":0.7480000257492065},{"id":"https://openalex.org/keywords/lysosomal-storage-disorders","display_name":"lysosomal storage disease","score":0.953000009059906},{"id":"https://openalex.org/keywords/lysosomes","display_name":"lysosomes","score":0.9399999976158142},{"id":"https://openalex.org/keywords/glucose-metabolism","display_name":"glucose metabolism","score":0.7160000205039978},{"id":"https://openalex.org/keywords/brain-development","display_name":"brain development","score":0.906000018119812},{"id":"https://openalex.org/keywords/mitochondrial-dysfunction","display_name":"mitochondrial dysfunction","score":0.7670000195503235},{"id":"https://openalex.org/keywords/neurotransmitter-homeostasis","display_name":"neurotransmitter homeostasis","score":0.9850000143051147},{"id":"https://openalex.org/keywords/neuronal-damage","display_name":"neuronal injury","score":0.6729999780654907},{"id":"https://openalex.org/keywords/lysosomal-dysfunction","display_name":"lysosomal dysfunction","score":0.503000020980835}],"concepts":[{"id":"https://openalex.org/C28859421","wikidata":"https://www.wikidata.org/wiki/Q39572","display_name":"Mitochondrion","level":2,"score":0.5865693092346191},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.5817148089408875},{"id":"https://openalex.org/C168240541","wikidata":"https://www.wikidata.org/wiki/Q45948","display_name":"Organelle","level":2,"score":0.5677278637886047},{"id":"https://openalex.org/C2986317502","wikidata":"https://www.wikidata.org/wiki/Q570714","display_name":"Energy metabolism","level":2,"score":0.5609632730484009},{"id":"https://openalex.org/C62231903","wikidata":"https://www.wikidata.org/wiki/Q1057","display_name":"Metabolism","level":2,"score":0.5500940680503845},{"id":"https://openalex.org/C2780216420","wikidata":"https://www.wikidata.org/wiki/Q83330","display_name":"Lysosome","level":3,"score":0.546514093875885},{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.5133408904075623},{"id":"https://openalex.org/C2776219046","wikidata":"https://www.wikidata.org/wiki/Q162657","display_name":"Neurotransmitter","level":3,"score":0.48016518354415894},{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.4553558826446533},{"id":"https://openalex.org/C4733338","wikidata":"https://www.wikidata.org/wiki/Q2792936","display_name":"Lipid metabolism","level":2,"score":0.42734724283218384},{"id":"https://openalex.org/C200170125","wikidata":"https://www.wikidata.org/wiki/Q969316","display_name":"Neurotransmission","level":3,"score":0.42371371388435364},{"id":"https://openalex.org/C529278444","wikidata":"https://www.wikidata.org/wiki/Q47273","display_name":"Central nervous system","level":2,"score":0.37490469217300415},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.36894840002059937},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.16723421216011047},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.15230721235275269},{"id":"https://openalex.org/C170493617","wikidata":"https://www.wikidata.org/wiki/Q208467","display_name":"Receptor","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":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004734","descriptor_name":"Energy Metabolism","qualifier_ui":"Q000502","qualifier_name":"physiology","is_major_topic":false},{"descriptor_ui":"D004734","descriptor_name":"Energy Metabolism","qualifier_ui":"Q000502","qualifier_name":"physiology","is_major_topic":false},{"descriptor_ui":"D004734","descriptor_name":"Energy Metabolism","qualifier_ui":"Q000502","qualifier_name":"physiology","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008247","descriptor_name":"Lysosomes","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008247","descriptor_name":"Lysosomes","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008247","descriptor_name":"Lysosomes","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D016464","descriptor_name":"Lysosomal Storage Diseases","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D016464","descriptor_name":"Lysosomal Storage Diseases","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D016464","descriptor_name":"Lysosomal Storage Diseases","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.1111/jnc.14577","doi":"https://doi.org/10.1111/jnc.14577","is_oa":false,"landing_page_url":"https://doi.org/10.1111/jnc.14577","pdf_url":null,"source":{"id":"https://openalex.org/S135837084","display_name":"Journal of Neurochemistry","issn_l":"0022-3042","issn":["0022-3042","1471-4159"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-d","doyens","jufo-2","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Neurochemistry","raw_type":"journal-article"},{"id":"pmid:30144055","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/30144055","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":"Journal of neurochemistry","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.5683000087738037,"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3"}],"sustainable_development_goals_aurora":[{"score":0.8700000047683716,"display_name":"Affordable and clean energy","id":"https://metadata.un.org/sdg/7"}],"study_designs":[],"awards":[],"funders":[{"id":"https://openalex.org/F4320322025","display_name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico","ror":"https://ror.org/03swz6y49"}],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":86,"referenced_works":["https://openalex.org/W24802232","https://openalex.org/W1494453148","https://openalex.org/W1529235958","https://openalex.org/W1583367908","https://openalex.org/W1607895012","https://openalex.org/W1678804523","https://openalex.org/W1780178978","https://openalex.org/W1799409770","https://openalex.org/W1940063962","https://openalex.org/W1940329105","https://openalex.org/W1953311373","https://openalex.org/W1967558353","https://openalex.org/W1968048563","https://openalex.org/W1970047246","https://openalex.org/W1971240747","https://openalex.org/W1973094968","https://openalex.org/W1979211500","https://openalex.org/W1988808060","https://openalex.org/W1989355184","https://openalex.org/W1992121643","https://openalex.org/W1993948951","https://openalex.org/W1994015846","https://openalex.org/W2005360742","https://openalex.org/W2010725039","https://openalex.org/W2011555635","https://openalex.org/W2014323841","https://openalex.org/W2025478209","https://openalex.org/W2027162428","https://openalex.org/W2031703986","https://openalex.org/W2035831296","https://openalex.org/W2037237112","https://openalex.org/W2040364482","https://openalex.org/W2043403270","https://openalex.org/W2043830216","https://openalex.org/W2046110239","https://openalex.org/W2046148946","https://openalex.org/W2051886358","https://openalex.org/W2057678559","https://openalex.org/W2060602529","https://openalex.org/W2063780783","https://openalex.org/W2064796242","https://openalex.org/W2064978465","https://openalex.org/W2072810901","https://openalex.org/W2074822339","https://openalex.org/W2079203448","https://openalex.org/W2082276782","https://openalex.org/W2082307109","https://openalex.org/W2103457363","https://openalex.org/W2105769353","https://openalex.org/W2112302648","https://openalex.org/W2113914303","https://openalex.org/W2115845435","https://openalex.org/W2116123511","https://openalex.org/W2116210148","https://openalex.org/W2116363928","https://openalex.org/W2134206147","https://openalex.org/W2136735437","https://openalex.org/W2139547290","https://openalex.org/W2149785020","https://openalex.org/W2151985574","https://openalex.org/W2152220015","https://openalex.org/W2156732679","https://openalex.org/W2170620150","https://openalex.org/W2189599899","https://openalex.org/W2221455105","https://openalex.org/W2296060917","https://openalex.org/W2326199983","https://openalex.org/W2336942037","https://openalex.org/W2398648256","https://openalex.org/W2416498602","https://openalex.org/W2419781546","https://openalex.org/W2464503524","https://openalex.org/W2509698629","https://openalex.org/W2550502192","https://openalex.org/W2575523348","https://openalex.org/W2584889633","https://openalex.org/W2623302327","https://openalex.org/W2643954325","https://openalex.org/W2739924689","https://openalex.org/W2770283973","https://openalex.org/W2774658967","https://openalex.org/W2784667865","https://openalex.org/W2789667300","https://openalex.org/W2790245192","https://openalex.org/W2809995090","https://openalex.org/W4210979710"],"related_works":["https://openalex.org/W2039857764","https://openalex.org/W2611958649","https://openalex.org/W2575523348","https://openalex.org/W2531350834","https://openalex.org/W2913425776","https://openalex.org/W3213275336","https://openalex.org/W2219723129","https://openalex.org/W4411273060","https://openalex.org/W4298034093","https://openalex.org/W2162470430"],"abstract_inverted_index":{"The":[0],"brain":[1,10,20,41,54,103],"has":[2],"a":[3,112],"very":[4],"high":[5],"requirement":[6],"for":[7,29,32,40,59,139],"energy.":[8],"Adult":[9],"relies":[11],"on":[12],"glucose":[13,28],"as":[14,25,27],"an":[15],"energy":[16,30,46,116],"substrate,":[17],"whereas":[18],"developing":[19],"can":[21,86],"utilize":[22],"alternative":[23],"substrates":[24],"well":[26],"and":[31,37,53,56,72,78,118,133,137],"the":[33,45,76,100,119,154],"biosynthesis":[34],"of":[35,67,102,115,128,153],"lipids":[36,74],"proteins":[38,71],"required":[39,47],"development.":[42],"Metabolism":[43],"provides":[44,111],"to":[48,131],"support":[49],"all":[50],"cellular":[51],"functions":[52],"development":[55],"building":[57],"blocks":[58],"macromolecules.":[60],"Lysosomes":[61],"are":[62,135],"organelles":[63],"involved":[64,122],"in":[65,75,99,105,123,125,142],"breakdown":[66],"biological":[68],"compounds":[69],"including":[70],"complex":[73],"body":[77],"brain.":[79,126],"Recent":[80],"studies":[81,94],"suggest":[82],"that":[83],"lysosomal":[84,106,144],"dysfunction":[85,98,141],"damage":[87,104],"neurons":[88],"and/or":[89],"alter":[90],"neurotransmitter":[91],"homeostasis.":[92],"Several":[93],"also":[95],"implicate":[96],"mitochondrial":[97,140],"pathophysiology":[101],"storage":[107,145],"diseases.":[108],"This":[109,149],"manuscript":[110],"brief":[113],"review":[114],"metabolism":[117,124,132],"key":[120],"pathways":[121],"Roles":[127],"lysosomes":[129],"related":[130],"neurotransmission":[134],"discussed,":[136],"evidence":[138],"several":[143],"diseases":[146],"is":[147,151],"presented.":[148],"article":[150],"part":[152],"Special":[155],"Issue":[156],"\"Lysosomal":[157],"Storage":[158],"Disorders\".":[159]},"counts_by_year":[{"year":2026,"cited_by_count":4},{"year":2025,"cited_by_count":5},{"year":2024,"cited_by_count":2},{"year":2023,"cited_by_count":6},{"year":2021,"cited_by_count":7},{"year":2020,"cited_by_count":3},{"year":2019,"cited_by_count":2}],"updated_date":"2026-10-27T15:51:10.671819","created_date":"2018-08-31T00:00:00"}