{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:46:14Z","timestamp":1786977974001,"version":"build-2736575974"},"reference-count":234,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T00:00:00Z","timestamp":1765324800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T00:00:00Z","timestamp":1765324800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Pathological ectopic calcification of soft tissues can arise from reduced or absent levels of inorganic pyrophosphate (PPi), a key inhibitor of calcium hydroxyapatite deposition in soft connective tissues. The role of PPi in regulating mineralization has been recognized for decades, thanks to the pivotal work of Herbert Fleisch and colleagues; and its clinical relevance has been underscored by the identification of hereditary metabolic disorders, collectively termed PPi deficiency syndromes. These are caused by pathogenic variants in the essential genes for maintaining PPi homeostasis: ATP\u2010binding cassette subfamily C member 6 (\n                    <jats:italic>ABCC6<\/jats:italic>\n                    ), ectonucleotide pyrophosphate phosphodiesterase 1 (\n                    <jats:italic>ENPP1<\/jats:italic>\n                    ), progressive ankylosis protein (\n                    <jats:italic>ANK<\/jats:italic>\n                    ), tissue\u2010nonspecific alkaline phosphatase (\n                    <jats:italic>ALPL<\/jats:italic>\n                    ),\n                    <jats:italic>CD73<\/jats:italic>\n                    , and\n                    <jats:italic>CD39<\/jats:italic>\n                    . In recent years, abnormalities in lipid metabolism have been reported in these monogenic conditions. However, a common understanding of these alterations has yet to be established. This review provides an overview of the pathophysiology of PPi deficiency syndromes\u2014pseudoxanthoma elasticum, generalized arterial calcification of infancy, arterial calcification due to CD73 deficiency, ankylosis, and Hutchinson\u2010Gilford progeria syndrome\u2014highlighting the lipid metabolism alterations in cells, animal models, and patients. We explore the evidence for a potential role of PPi\u2010regulating proteins in lipid metabolic pathways to demonstrate that lipid alterations are not coincidental but entail opportunities for future research and for potential therapeutic interventions.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70129","type":"journal-article","created":{"date-parts":[[2025,12,11]],"date-time":"2025-12-11T07:24:04Z","timestamp":1765437844000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Lipid Metabolism Alterations in Hereditary Inorganic Pyrophosphate Deficiency Syndromes: A Narrative Review\u00a0of Insights and Controversies"],"prefix":"10.1002","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6413-8215","authenticated-orcid":false,"given":"Robbe","family":"Derudder","sequence":"first","affiliation":[{"name":"Center for Medical Genetics, Ghent University Hospital Ghent University  Ghent Belgium"},{"name":"Department of Biomolecular Medicine Ghent University  Ghent Belgium"},{"name":"Ghent Ectopic Mineralization Research Group  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier M.","family":"Vanakker","sequence":"additional","affiliation":[{"name":"Center for Medical Genetics, Ghent University Hospital Ghent University  Ghent Belgium"},{"name":"Department of Biomolecular Medicine Ghent University  Ghent Belgium"},{"name":"Ghent Ectopic Mineralization Research Group  Ghent Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,10]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2022.01.012"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-323-91599-1.00003-1"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dsx.2019.03.033"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.P008268"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2008.12.006"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1517\/14622416.2.1.51"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1319582110"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1161\/ATVBAHA.114.304017"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20040465"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2018.09.014"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.11.020"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-016-0574-z"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.4528"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.289.5477.265"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1008884"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2017.00161"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/biom10121648"},{"issue":"5","key":"e_1_2_12_19_1","first-page":"767","article-title":"Alkaline Phosphatase (Tissue\u2010Nonspecific Isoenzyme) is a Phosphoethanolamine and Pyridoxal\u20105\u2032\u2010Phosphate Ectophosphatase: Normal and Hypophosphatasia Fibroblast Study","volume":"47","author":"Fedde K. 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