{"id":"https://openalex.org/W4392454301","doi":"https://doi.org/10.3233/jhd-231511","title":"Alterations in Cerebrospinal Fluid Urea Occur in Late Manifest Huntington\u2019s Disease","display_name":"Alterations in Cerebrospinal Fluid Urea Occur in Late Manifest Huntington\u2019s Disease","publication_year":2024,"publication_date":"2024-03-05","ids":{"openalex":"https://openalex.org/W4392454301","doi":"https://doi.org/10.3233/jhd-231511","pmid":"https://pubmed.ncbi.nlm.nih.gov/38461512","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/11238568"},"language":"en","primary_location":{"id":"doi:10.3233/jhd-231511","doi":"https://doi.org/10.3233/jhd-231511","is_oa":false,"landing_page_url":"https://doi.org/10.3233/jhd-231511","pdf_url":null,"source":{"id":"https://openalex.org/S2764536931","display_name":"Journal of Huntington s Disease","issn_l":"1879-6397","issn":["1879-6397","1879-6400"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-d","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2"],"host_organization":"https://openalex.org/P4310318577","host_organization_name":"IOS Press","host_organization_lineage":["https://openalex.org/P4310318577"],"host_organization_lineage_names":["IOS Press"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Huntington's Disease","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC11238568/pdf/nihms-2005524.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5087490035","display_name":"Anna C. Pfalzer","orcid":"https://orcid.org/0000-0001-8317-3318","observed_orcids":["https://orcid.org/0000-0001-8317-3318"]},"institutions":[{"id":"https://openalex.org/I901861585","display_name":"Vanderbilt University Medical Center","ror":"https://ror.org/05dq2gs74","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210162197","https://openalex.org/I901861585"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Anna C. Pfalzer","raw_affiliation_strings":["Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA","institution_ids":["https://openalex.org/I901861585"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5071555088","display_name":"Shuhei Shiino","orcid":"https://orcid.org/0009-0005-0845-7173","observed_orcids":["https://orcid.org/0009-0005-0845-7173"]},"institutions":[{"id":"https://openalex.org/I901861585","display_name":"Vanderbilt University Medical Center","ror":"https://ror.org/05dq2gs74","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210162197","https://openalex.org/I901861585"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Shuhei Shiino","raw_affiliation_strings":["Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA","institution_ids":["https://openalex.org/I901861585"]}]},{"author_position":"middle","author":{"id":null,"display_name":"James Silverman","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I901861585","display_name":"Vanderbilt University Medical Center","ror":"https://ror.org/05dq2gs74","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210162197","https://openalex.org/I901861585"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"James Silverman","raw_affiliation_strings":["Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA","institution_ids":["https://openalex.org/I901861585"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5070957190","display_name":"Simona G. Codreanu","orcid":"https://orcid.org/0000-0003-4435-812X","observed_orcids":["https://orcid.org/0000-0003-4435-812X"]},"institutions":[{"id":"https://openalex.org/I200719446","display_name":"Vanderbilt University","ror":"https://ror.org/02vm5rt34","country_code":"US","type":"education","lineage":["https://openalex.org/I200719446"]},{"id":"https://openalex.org/I4210121737","display_name":"Virginia Innovation Partnership Corporation","ror":"https://ror.org/02y1rjh68","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I4210121737"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Simona G. Codreanu","raw_affiliation_strings":["Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","E-mail:"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","institution_ids":["https://openalex.org/I200719446","https://openalex.org/I4210121737"]},{"raw_affiliation_string":"E-mail:","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5063940912","display_name":"Stacy D. Sherrod","orcid":"https://orcid.org/0000-0002-2346-230X","observed_orcids":["https://orcid.org/0000-0002-2346-230X"]},"institutions":[{"id":"https://openalex.org/I200719446","display_name":"Vanderbilt University","ror":"https://ror.org/02vm5rt34","country_code":"US","type":"education","lineage":["https://openalex.org/I200719446"]},{"id":"https://openalex.org/I4210121737","display_name":"Virginia Innovation Partnership Corporation","ror":"https://ror.org/02y1rjh68","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I4210121737"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Stacy D. Sherrod","raw_affiliation_strings":["Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","E-mail:"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","institution_ids":["https://openalex.org/I200719446","https://openalex.org/I4210121737"]},{"raw_affiliation_string":"E-mail:","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5026144955","display_name":"JOHN A. JUN. MCLEAN","orcid":"https://orcid.org/0000-0001-8918-6419","observed_orcids":["https://orcid.org/0000-0001-8918-6419"]},"institutions":[{"id":"https://openalex.org/I200719446","display_name":"Vanderbilt University","ror":"https://ror.org/02vm5rt34","country_code":"US","type":"education","lineage":["https://openalex.org/I200719446"]},{"id":"https://openalex.org/I4210121737","display_name":"Virginia Innovation Partnership Corporation","ror":"https://ror.org/02y1rjh68","country_code":"US","type":"nonprofit","lineage":["https://openalex.org/I4210121737"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"John A. McLean","raw_affiliation_strings":["Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","E-mail:"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Chemistry and Center for Innovative Technology, Vanderbilt University, Nashville, TN, USA","institution_ids":["https://openalex.org/I200719446","https://openalex.org/I4210121737"]},{"raw_affiliation_string":"E-mail:","institution_ids":[]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5064135529","display_name":"Daniel O. Claassen","orcid":"https://orcid.org/0000-0002-9853-4902","observed_orcids":["https://orcid.org/0000-0002-9853-4902"]},"institutions":[{"id":"https://openalex.org/I901861585","display_name":"Vanderbilt University Medical Center","ror":"https://ror.org/05dq2gs74","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210162197","https://openalex.org/I901861585"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Daniel O. Claassen","raw_affiliation_strings":["Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA","institution_ids":["https://openalex.org/I901861585"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5087490035"],"corresponding_institution_ids":["https://openalex.org/I901861585"],"apc_list":{"value":4150,"currency":"USD","value_usd":4150},"apc_paid":null,"fwci":1.1529,"has_fulltext":true,"cited_by_count":7,"citation_normalized_percentile":{"value":0.75184969,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":98},"biblio":{"volume":"13","issue":"1","first_page":"103","last_page":"111"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10949","display_name":"Genetic Neurodegenerative Diseases","score":0.9937000274658203,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T10949","display_name":"Genetic Neurodegenerative Diseases","score":0.9937000274658203,"subfield":{"id":"https://openalex.org/subfields/2804","display_name":"Cellular and Molecular Neuroscience"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.002899999963119626,"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/T10836","display_name":"Metabolomics and Mass Spectrometry Studies","score":0.0005000000237487257,"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"}}],"keywords":[{"id":"https://openalex.org/keywords/huntington-s-disease","display_name":"Huntington's disease","score":1.0},{"id":"https://openalex.org/keywords/metabolomics","display_name":"metabolomics","score":0.6209999918937683},{"id":"https://openalex.org/keywords/disease-stage","display_name":"disease stage","score":0.597000002861023},{"id":"https://openalex.org/keywords/cellular-energy-metabolism","display_name":"cellular energy metabolism","score":0.7279999852180481},{"id":"https://openalex.org/keywords/mutant-huntingtin","display_name":"mutant huntingtin","score":0.7450000047683716},{"id":"https://openalex.org/keywords/citrulline","display_name":"citrulline","score":0.8019999861717224},{"id":"https://openalex.org/keywords/ornithine","display_name":"ornithine","score":0.9900000095367432},{"id":"https://openalex.org/keywords/l-arginine","display_name":"L-arginine","score":0.9340000152587891},{"id":"https://openalex.org/keywords/neurodegeneration","display_name":"neurodegeneration","score":0.7250000238418579}],"concepts":[{"id":"https://openalex.org/C2780647506","wikidata":"https://www.wikidata.org/wiki/Q190564","display_name":"Huntington's disease","level":3,"score":0.7626532316207886},{"id":"https://openalex.org/C2780365088","wikidata":"https://www.wikidata.org/wiki/Q48318","display_name":"Urea","level":2,"score":0.7407686710357666},{"id":"https://openalex.org/C77061444","wikidata":"https://www.wikidata.org/wiki/Q595994","display_name":"Urea cycle","level":4,"score":0.7355412840843201},{"id":"https://openalex.org/C2776796294","wikidata":"https://www.wikidata.org/wiki/Q410198","display_name":"Ornithine","level":4,"score":0.701705276966095},{"id":"https://openalex.org/C2781427258","wikidata":"https://www.wikidata.org/wiki/Q2620886","display_name":"Huntingtin","level":4,"score":0.6842644214630127},{"id":"https://openalex.org/C2777477808","wikidata":"https://www.wikidata.org/wiki/Q407595","display_name":"Metabolite","level":2,"score":0.6432174444198608},{"id":"https://openalex.org/C2779651940","wikidata":"https://www.wikidata.org/wiki/Q54196","display_name":"Cerebrospinal fluid","level":2,"score":0.6089658737182617},{"id":"https://openalex.org/C21565614","wikidata":"https://www.wikidata.org/wiki/Q12149006","display_name":"Metabolomics","level":2,"score":0.5817166566848755},{"id":"https://openalex.org/C2777468819","wikidata":"https://www.wikidata.org/wiki/Q173670","display_name":"Arginine","level":3,"score":0.5799815654754639},{"id":"https://openalex.org/C2910011555","wikidata":"https://www.wikidata.org/wiki/Q2620886","display_name":"Huntingtin Protein","level":5,"score":0.5256063938140869},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.5164406299591064},{"id":"https://openalex.org/C2779129087","wikidata":"https://www.wikidata.org/wiki/Q408641","display_name":"Citrulline","level":4,"score":0.5029007792472839},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.4925321042537689},{"id":"https://openalex.org/C164007495","wikidata":"https://www.wikidata.org/wiki/Q24721374","display_name":"Arginase","level":4,"score":0.4908803105354309},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4619671702384949},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.37740015983581543},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.28915566205978394},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.2818111777305603},{"id":"https://openalex.org/C60644358","wikidata":"https://www.wikidata.org/wiki/Q128570","display_name":"Bioinformatics","level":1,"score":0.18431687355041504},{"id":"https://openalex.org/C515207424","wikidata":"https://www.wikidata.org/wiki/Q8066","display_name":"Amino acid","level":2,"score":0.0}],"mesh":[{"descriptor_ui":"D000071058","descriptor_name":"Huntingtin Protein","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000071058","descriptor_name":"Huntingtin Protein","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","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":"D006816","descriptor_name":"Huntington Disease","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D014508","descriptor_name":"Urea","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D018450","descriptor_name":"Disease Progression","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":3,"locations":[{"id":"doi:10.3233/jhd-231511","doi":"https://doi.org/10.3233/jhd-231511","is_oa":false,"landing_page_url":"https://doi.org/10.3233/jhd-231511","pdf_url":null,"source":{"id":"https://openalex.org/S2764536931","display_name":"Journal of Huntington s Disease","issn_l":"1879-6397","issn":["1879-6397","1879-6400"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-d","jufo-1","ki-jl-1","medline","norway-1","poland-70","russia-white-list-2"],"host_organization":"https://openalex.org/P4310318577","host_organization_name":"IOS Press","host_organization_lineage":["https://openalex.org/P4310318577"],"host_organization_lineage_names":["IOS Press"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Huntington's Disease","raw_type":"journal-article"},{"id":"pmid:38461512","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/38461512","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 Huntington's disease","raw_type":"Journal Article"},{"id":"pmh:oai:pubmedcentral.nih.gov:11238568","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/11238568","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC11238568/pdf/nihms-2005524.pdf","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":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":"J Huntingtons Dis","raw_type":"Text"}],"best_oa_location":{"id":"pmh:oai:pubmedcentral.nih.gov:11238568","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/11238568","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC11238568/pdf/nihms-2005524.pdf","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":null,"license_id":null,"version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":"J Huntingtons Dis","raw_type":"Text"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9537000060081482,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[{"id":"https://openalex.org/G2615776415","display_name":null,"funder_award_id":"UL1TR002243","funder_id":"https://openalex.org/F4320316438","funder_display_name":"Georgia Clinical and Translational Science Alliance"},{"id":"https://openalex.org/G3124268028","display_name":"Understanding metabolic changes associated with chronic manganese exposure and Alzheimer's Disease","funder_award_id":"R03NS125243","funder_id":"https://openalex.org/F4320337359","funder_display_name":"National Institute of Neurological Disorders and Stroke"},{"id":"https://openalex.org/G4725597257","display_name":"Overall: Eunice Kennedy Shriver Intellectual and Developmental Disabilities Research Center at Vanderbilt","funder_award_id":"P50HD103537","funder_id":"https://openalex.org/F4320337611","funder_display_name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development"},{"id":"https://openalex.org/G5225572034","display_name":"MICA: HD-CSF: Studying cerebrospinal fluid to understand key CNS pathobiological targets in Huntington's disease","funder_award_id":"MR/M008592/1","funder_id":"https://openalex.org/F4320334626","funder_display_name":"Medical Research Council"},{"id":"https://openalex.org/G5580019600","display_name":null,"funder_award_id":"TR002243","funder_id":"https://openalex.org/F4320337472","funder_display_name":"National Center for Advancing Translational Sciences"},{"id":"https://openalex.org/G659731888","display_name":null,"funder_award_id":"UL1\u2010TR002243","funder_id":"https://openalex.org/F4320309151","funder_display_name":"Vanderbilt University"},{"id":"https://openalex.org/G9000872578","display_name":"Vanderbilt Institute for Clinical and Translational Research (VICTR)","funder_award_id":"UL1TR002243","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"}],"funders":[{"id":"https://openalex.org/F4320308687","display_name":"CHDI Foundation","ror":"https://ror.org/046eh8t80"},{"id":"https://openalex.org/F4320309151","display_name":"Vanderbilt University","ror":"https://ror.org/02vm5rt34"},{"id":"https://openalex.org/F4320316438","display_name":"Georgia Clinical and Translational Science Alliance","ror":"https://ror.org/034hw7m59"},{"id":"https://openalex.org/F4320320286","display_name":"University College London","ror":"https://ror.org/02jx3x895"},{"id":"https://openalex.org/F4320332161","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88"},{"id":"https://openalex.org/F4320334626","display_name":"Medical Research Council","ror":"https://ror.org/03x94j517"},{"id":"https://openalex.org/F4320337359","display_name":"National Institute of Neurological Disorders and Stroke","ror":"https://ror.org/01s5ya894"},{"id":"https://openalex.org/F4320337472","display_name":"National Center for Advancing Translational Sciences","ror":"https://ror.org/04pw6fb54"},{"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/W4392454301.pdf","grobid_xml":"https://content.openalex.org/works/W4392454301.grobid-xml"},"referenced_works_count":28,"referenced_works":["https://openalex.org/W1840108306","https://openalex.org/W1994536298","https://openalex.org/W2025658241","https://openalex.org/W2063028213","https://openalex.org/W2081840097","https://openalex.org/W2084375915","https://openalex.org/W2121642311","https://openalex.org/W2149248612","https://openalex.org/W2194890007","https://openalex.org/W2520784563","https://openalex.org/W2566732839","https://openalex.org/W2588591933","https://openalex.org/W2770942592","https://openalex.org/W2774354480","https://openalex.org/W2802834706","https://openalex.org/W2974361058","https://openalex.org/W3033538229","https://openalex.org/W3080389087","https://openalex.org/W3108234709","https://openalex.org/W3118523284","https://openalex.org/W3122170578","https://openalex.org/W3125488374","https://openalex.org/W3146010890","https://openalex.org/W3160056147","https://openalex.org/W3205518935","https://openalex.org/W4210504143","https://openalex.org/W4283170294","https://openalex.org/W4285217899"],"related_works":["https://openalex.org/W2774354480","https://openalex.org/W2921441681","https://openalex.org/W3108234709","https://openalex.org/W1840108306","https://openalex.org/W7066580006","https://openalex.org/W2590107639","https://openalex.org/W3198396429","https://openalex.org/W4390795455","https://openalex.org/W2200373289","https://openalex.org/W3048269128"],"abstract_inverted_index":{"Background:":[0],"Huntington's":[1],"disease":[2,51],"(HD)":[3],"is":[4],"a":[5,33,88],"neurodegenerative":[6],"disorder":[7],"caused":[8],"by":[9],"expanded":[10],"cytosine-adenine-guanine":[11],"(CAG)":[12],"repeats":[13],"in":[14,19,37,83,126,136,149,157,175,186],"the":[15,20,48,58,71,158,176],"Huntingtin":[16],"gene,":[17],"resulting":[18],"production":[21],"of":[22,44,76,92,103],"mutant":[23],"huntingtin":[24],"proteins":[25],"(mHTT).":[26],"Previous":[27],"research":[28],"has":[29],"identified":[30],"urea":[31,54,77,138,140,145,159],"as":[32],"key":[34],"metabolite":[35],"elevated":[36,148],"HD":[38,45,113,177],"animal":[39],"models":[40],"and":[41,53,74,109,115,163,179],"postmortem":[42],"tissues":[43],"patients.":[46],"However,":[47],"relationship":[49],"between":[50],"course":[52],"elevations,":[55],"along":[56],"with":[57],"molecular":[59,72],"mechanisms":[60],"responsible":[61],"for":[62],"these":[63,180],"disturbances":[64,73],"remain":[65],"unknown.":[66],"Objective:":[67],"To":[68],"better":[69],"understand":[70],"timing":[75],"cycle":[78],"metabolism":[79],"across":[80],"different":[81],"stages":[82,102],"HD.":[84],"Methods:":[85],"We":[86],"completed":[87],"global":[89],"metabolomic":[90],"profile":[91],"cerebrospinal":[93],"fluid":[94],"(CSF)":[95],"from":[96],"individuals":[97],"who":[98],"were":[99,123,142,154],"at":[100],"several":[101],"disease:":[104],"pre-manifest":[105],"(PRE),":[106],"manifest":[107,111],"(MAN),":[108],"late":[110,174],"(LATE)":[112],"participants,":[114],"compared":[116,129],"to":[117,130],"controls.":[118],"Results:":[119],"Approximately":[120],"500":[121],"metabolites":[122,141,160],"significantly":[124,147],"altered":[125],"PRE":[127],"participants":[128,151],"controls,":[131],"although":[132],"no":[133,155],"significant":[134],"differences":[135],"CSF":[137,144,171],"or":[139],"observed.":[143],"was":[146],"LATE":[150],"only.":[152],"There":[153],"changes":[156,181],"citrulline,":[161],"ornithine,":[162],"arginine.":[164],"Conclusions:":[165],"Overall,":[166],"our":[167],"study":[168],"confirms":[169],"that":[170],"elevations":[172],"occur":[173],"course,":[178],"may":[182],"reflect":[183],"accumulating":[184],"deficits":[185],"cellular":[187],"energy":[188],"metabolism.":[189]},"counts_by_year":[{"year":2026,"cited_by_count":4},{"year":2025,"cited_by_count":2},{"year":2024,"cited_by_count":1}],"updated_date":"2026-11-03T10:50:19.383937","created_date":"2024-03-06T00:00:00"}
