{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T11:00:39Z","timestamp":1784372439826,"version":"3.55.0"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T00:00:00Z","timestamp":1735257600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003092","name":"Diabetes Fonds","doi-asserted-by":"publisher","award":["2017.82.004"],"award-info":[{"award-number":["2017.82.004"]}],"id":[{"id":"10.13039\/501100003092","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","award":["114024071"],"award-info":[{"award-number":["114024071"]}],"id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Hereditary fructose intolerance (HFI) is characterized by liver damage and a secondary defect in N\u2010linked glycosylation due to impairment of mannose phosphate isomerase (MPI). Mannose treatment has been shown to be an effective treatment in a primary defect in MPI (i.e., MPI\u2010CDG), which is also characterized by liver damage. Therefore, the aims of this study were to determine: (1) hepatic nucleotide sugar levels, and (2), the effect of mannose supplementation on hepatic nucleotide sugar levels and liver fat, in a mouse model for HFI. Aldolase B deficient mice (<jats:italic>Aldob<\/jats:italic><jats:sup><jats:italic>\u2212\/\u2212<\/jats:italic><\/jats:sup>) were treated for four weeks with 5% mannose via the drinking water and compared to <jats:italic>Aldob<\/jats:italic><jats:sup><jats:italic>\u2212\/<\/jats:italic>\u2212<\/jats:sup> mice and wildtype mice treated with regular drinking water. We found that hepatic GDP\u2010mannose and hepatic GDP\u2010fucose were lower in water\u2010treated <jats:italic>Aldob<\/jats:italic><jats:sup><jats:italic>\u2212\/<\/jats:italic>\u2212<\/jats:sup> mice when compared to water\u2010treated wildtype mice (<jats:italic>p<\/jats:italic>\u2009=\u20090.002 and <jats:italic>p<\/jats:italic>\u2009=\u20090.002, respectively), consistent with impaired N\u2010linked glycosylation. Of interest, multiple other hepatic nucleotide sugars not involved in N\u2010linked glycosylation, such as hepatic UDP\u2010glucuronic acid, UDP\u2010xylose, CMP\u2010<jats:italic>N<\/jats:italic>\u2010acetyl\u2010beta\u2010neuraminic acid, and CDP\u2010ribitol (<jats:italic>p<\/jats:italic>\u2009=\u20090.002, <jats:italic>p<\/jats:italic>\u2009=\u20090.003, <jats:italic>p<\/jats:italic>\u2009=\u20090.002, <jats:italic>p<\/jats:italic>\u2009=\u20090.002), were found to have altered levels as well. However, mannose treatment did not correct the reduction in hepatic GDP\u2010mannose levels, nor was liver fat affected. <jats:italic>Aldob<\/jats:italic><jats:sup><jats:italic>\u2212\/<\/jats:italic>\u2212<\/jats:sup> mice are characterized by hepatic nucleotide sugar abnormalities, but these were not abrogated by mannose treatment. Future studies are needed to identify the underlying mechanisms responsible for the abnormal hepatic nucleotide sugar pattern and intrahepatic lipid accumulation in HFI.<\/jats:p><jats:p><jats:bold>Trial Registration:<\/jats:bold> PCT ID: PCTE0000340, this animal experiment is registered at (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"https:\/\/preclinicaltrials.eu\/\">https:\/\/preclinicaltrials.eu\/<\/jats:ext-link>).<\/jats:p>","DOI":"10.1002\/jimd.12836","type":"journal-article","created":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T05:38:08Z","timestamp":1735277888000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Aldolase B Deficient Mice Are Characterized by Hepatic Nucleotide Sugar Abnormalities"],"prefix":"10.1002","volume":"48","author":[{"given":"Am\u00e9e M.","family":"Buziau","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Division of Endocrinology and Metabolic Disease Maastricht University Medical Center+  Maastricht The Netherlands"},{"name":"Cardiovascular Research Institute Maastricht (CARIM) Maastricht University  Maastricht The Netherlands"},{"name":"Department of Internal Medicine, Division of General Internal Medicine, Laboratory for Metabolism and Vascular Medicine Maastricht University  Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk J.","family":"Lefeber","sequence":"additional","affiliation":[{"name":"Translational Metabolic Laboratory, Department of Human Genetics Radboud University Medical Center  Nijmegen The Netherlands"},{"name":"Department of Neurology, Donders Institute for Brain, Cognition and Behavior Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Cassiman","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology\u2010Hepatology and Metabolic Center University Hospital Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Estela","family":"Rubio\u2010Gozalbo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Clinical Genetics Maastricht University Medical Center+  Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanneke","family":"Kwast","sequence":"additional","affiliation":[{"name":"Translational Metabolic Laboratory, Department of Human Genetics Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dean R.","family":"Tolan","sequence":"additional","affiliation":[{"name":"Department of Biology Boston University  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Casper G.","family":"Schalkwijk","sequence":"additional","affiliation":[{"name":"Cardiovascular Research Institute Maastricht (CARIM) Maastricht University  Maastricht The Netherlands"},{"name":"Department of Internal Medicine, Division of General Internal Medicine, Laboratory for Metabolism and Vascular Medicine Maastricht University  Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8229-3331","authenticated-orcid":false,"given":"Martijn C. G. J.","family":"Brouwers","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Division of Endocrinology and Metabolic Disease Maastricht University Medical Center+  Maastricht The Netherlands"},{"name":"Cardiovascular Research Institute Maastricht (CARIM) Maastricht University  Maastricht The Netherlands"},{"name":"Department of Internal Medicine Maastricht University Medical Center+  Maastricht The Netherlands"},{"name":"United for Metabolic Diseases  The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,12,27]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(56)92196-1"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000458107"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.33.10.837"},{"key":"e_1_2_10_5_1","first-page":"40","article-title":"Vorteile und Nachteile der Fructose in der Nahrung","volume":"37","author":"Gitzelmann R.","year":"1973","journal-title":"Paediatr Fortbildungsk Prax"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/mcp.M113.035600"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-3002(53)90062-6"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1978.02120310069014"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198309293091305"},{"issue":"6","key":"e_1_2_10_10_1","first-page":"599","article-title":"A Case of Fatal Hereditary Fructose Intolerance. Misleading Information of Formula Composition","volume":"36","author":"Von Ruecker A.","year":"1981","journal-title":"Helvetica Paediatrica Acta"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(68)90181-2"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.35.5.353"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.8.1.8299892"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2018.02.016"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2018-02795"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu11102397"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2019.02.019"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2023.100692"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-019-03348-2"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molmet.2024.101984"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199611000-00017"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmd2.12379"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm10132932"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-63123-2"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2007.08.126"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-016-0012-4"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.01.001"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI94427"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwu006"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwab106"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12265"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2005.10.033"},{"key":"e_1_2_10_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.32064"},{"key":"e_1_2_10_34_1","first-page":"599","volume-title":"Essentials of Glycobiology (Internet)","author":"Lefeber D. J.","year":"2022"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2014.06.021"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-009-1219-4"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0569-z"},{"key":"e_1_2_10_38_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R300025200"},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.metabol.2018.01.025"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.13-245514"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jimd.12836","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T10:08:41Z","timestamp":1754906921000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jimd.12836"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,27]]},"references-count":39,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1002\/jimd.12836"],"URL":"https:\/\/doi.org\/10.1002\/jimd.12836","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,27]]},"assertion":[{"value":"2024-10-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-11-25","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-12-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12836"}}