{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T13:04:59Z","timestamp":1784293499890,"version":"3.55.0"},"reference-count":22,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T00:00:00Z","timestamp":1554076800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T00:00:00Z","timestamp":1554076800000},"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":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>This work analyzes the thermogenic flux induced by the very long\u2010chain fatty acid (VLCFA) lignoceric acid (C24:0) in isolated peroxisomes.<\/jats:p>\n                  <jats:p>Specific metabolic alterations of peroxisomes are related to a variety of disorders, the most frequent one being the neurodegenerative inherited disease X\u2010linked adrenoleukodystrophy (X\u2010ALD). A peroxisomal transport protein is mutated in this disorder. Due to reduced catabolism and enhanced fatty acid (FA) elongation, VLCFA accumulates in plasma and in all tissues, contributing to the clinical manifestations of this disorder. During peroxisomal metabolism, heat is produced but it is considered lost. Instead, it is a form of energy that could play a role in molecular mechanisms of this pathology and other neurodegenerative disorders.<\/jats:p>\n                  <jats:p>The thermogenic flux induced by lignoceric acid (C24:0) was estimated by isothermal titration calorimetry in peroxisomes isolated from HepG2 cells and from fibroblasts obtained from patients with\u00a0X\u2010ALD and healthy subjects.<\/jats:p>\n                  <jats:p>Heat flux induced by lignoceric acid in HepG2 peroxisomes was exothermic, indicating normal peroxisomal metabolism. In X\u2010ALD peroxisomes the heat flux was endothermic, indicating the requirement of heat\/energy, possibly for cellular metabolism. In fibroblasts from healthy subjects, the effect was less pronounced than in HepG2, a kind of cell known to have greater FA metabolism than fibroblasts.<\/jats:p>\n                  <jats:p>Our hypothesis is that heat is not lost but it could act as an activator, for example on the heat\u2010sensitive pathway related to TRVP2 receptors. To investigate this hypothesis we focused on peroxisomal metabolism, considering that impaired heat generation could contribute to the development of peroxisomal neurodegenerative disorders.<\/jats:p>","DOI":"10.1002\/jcp.28467","type":"journal-article","created":{"date-parts":[[2019,4,1]],"date-time":"2019-04-01T08:49:30Z","timestamp":1554108570000},"page":"18344-18348","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Thermogenic flux induced by lignoceric acid in peroxisomes isolated from HepG2 cells and from X\u2010adrenoleukodystrophy and control fibroblasts"],"prefix":"10.1002","volume":"234","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5863-8993","authenticated-orcid":false,"given":"Anna","family":"Petroni","sequence":"first","affiliation":[{"name":"Biomedicine and Nutrition Research Network Milan Italy"},{"name":"Department of Pharmacological and Biomolecular Sciences University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3186-8860","authenticated-orcid":false,"given":"Rita","family":"Paroni","sequence":"additional","affiliation":[{"name":"Department of Health Science (DISS) University of Milan,\u00a0ASST Santi Paolo e Carlo Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Maria","family":"Aloisi","sequence":"additional","affiliation":[{"name":"Department of Physiology, Neuroscience, and Applied Physiology Unit University of Siena Siena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8808-3127","authenticated-orcid":false,"given":"Milena","family":"Blasevich","sequence":"additional","affiliation":[{"name":"Biomedicine and Nutrition Research Network Milan Italy"},{"name":"Department of Pharmacological and Biomolecular Sciences University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nabil","family":"Haman","sequence":"additional","affiliation":[{"name":"Department of Food, Enviromental and Nutritional Sciences (DeFENS) University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4998-308X","authenticated-orcid":false,"given":"Dimitrios","family":"Fessas","sequence":"additional","affiliation":[{"name":"Department of Food, Enviromental and Nutritional Sciences (DeFENS) University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,4,1]]},"reference":[{"key":"e_1_2_9_1_2_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2265.1998.00640.x"},{"key":"e_1_2_9_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2004.03.003"},{"key":"e_1_2_9_1_4_1","first-page":"512","article-title":"Adrenoleukodystrophy: Incidence, new mutation rate and results of extended family screening","volume":"49","author":"Bezman L.","year":"2001","journal-title":"Neurology"},{"key":"e_1_2_9_1_5_1","first-page":"248","article-title":"A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein\u2010dye binding","volume":"72","author":"Bradford M. 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