{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T11:26:31Z","timestamp":1787311591992,"version":"build-2736575974"},"reference-count":37,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,6,1]],"date-time":"2019-06-01T00:00:00Z","timestamp":1559347200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2019,1,14]],"date-time":"2019-01-14T00:00:00Z","timestamp":1547424000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism Reports"],"published-print":{"date-parts":[[2019,6]]},"DOI":"10.1016\/j.ymgmr.2019.100451","type":"journal-article","created":{"date-parts":[[2019,1,30]],"date-time":"2019-01-30T00:10:13Z","timestamp":1548807013000},"page":"100451","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":13,"special_numbering":"C","title":["Feasibility of cellular bioenergetics as a biomarker in porphyria patients"],"prefix":"10.1016","volume":"19","author":[{"given":"Balu","family":"Chacko","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matilda Lillian","family":"Culp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Bloomer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Phillips","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong-Fang","family":"Kuo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor","family":"Darley-Usmar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashwani K.","family":"Singal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgmr.2019.100451_bb0005","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1056\/NEJMra1608634","article-title":"Porphyria","volume":"377","author":"Bissell","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0010","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1007\/s12664-016-0698-0","article-title":"Hepatic porphyria: a narrative review","volume":"35","author":"Arora","year":"2016","journal-title":"Indian J. Gastroenterol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0015","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1016\/j.freeradbiomed.2011.06.011","article-title":"Oxidases and peroxidases in cardiovascular and lung disease: new concepts in reactive oxygen species signaling","volume":"51","author":"Al Ghouleh","year":"2011","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0020","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1016\/j.exphem.2011.05.003","article-title":"Abnormal mitoferrin-1 expression in patients with erythropoietic protoporphyria","volume":"39","author":"Wang","year":"2011","journal-title":"Exp. Hematol."},{"issue":"7","key":"10.1016\/j.ymgmr.2019.100451_bb0025","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1016\/j.bbamcr.2006.05.005","article-title":"Biosynthesis of heme in mammals","volume":"1763","author":"Ajioka","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0030","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.biocel.2014.03.032","article-title":"Acute intermittent porphyria causes hepatic mitochondrial energetic failure in a mouse model","volume":"51","author":"Homedan","year":"2014","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0035","doi-asserted-by":"crossref","first-page":"7835","DOI":"10.1074\/jbc.M114.547778","article-title":"Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria","volume":"289","author":"Chung","year":"2014","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0040","doi-asserted-by":"crossref","first-page":"1420","DOI":"10.1002\/hep.22486","article-title":"Hepatitis C virus-induced oxidative stress suppresses hepcidin expression through increased histone deacetylase activity","volume":"48","author":"Miura","year":"2008","journal-title":"Hepatology"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0045","doi-asserted-by":"crossref","first-page":"5015","DOI":"10.1093\/hmg\/ddv222","article-title":"Mitochondrial energetic defects in muscle and brain of a Hmbs\u2212\/\u2212 mouse model of acute intermittent porphyria","volume":"24","author":"Homedan","year":"2015","journal-title":"Hum. Mol. Genet."},{"issue":"1992","key":"10.1016\/j.ymgmr.2019.100451_bb0050","first-page":"226","article-title":"5-aminolevulinic acid-induced alterations of oxidative metabolism in sedentary and exercise-trained rats","volume":"72","author":"Pereira","year":"1985","journal-title":"J. Appl. Physiol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0055","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1007\/s11306-017-1305-9","article-title":"Urinary metabolic profiling of asymptomatic acute intermittent porphyria using a rule-mining-based algorithm","volume":"14","author":"Luck","year":"2018","journal-title":"Metabolomics: Offl. J. Metabolomic Soc."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0060","doi-asserted-by":"crossref","first-page":"3003","DOI":"10.1089\/ars.2011.3921","article-title":"Mitochondrial mayhem: the mitochondrion as a modulator of iron metabolism and its role in disease","volume":"15","author":"Huang","year":"2011","journal-title":"Antioxid. Redox Signal."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0065","doi-asserted-by":"crossref","first-page":"1485","DOI":"10.1515\/hsz-2012-0198","article-title":"Integration of cellular bioenergetics with mitochondrial quality control and autophagy","volume":"393","author":"Hill","year":"2012","journal-title":"Biol. Chem."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0070","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.redox.2017.05.017","article-title":"Mitochondria-meditated pathways of organ failure upon inflammation","volume":"13","author":"Kozlov","year":"2017","journal-title":"Redox Biol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0075","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1186\/s13054-017-1691-4","article-title":"Time course of blood lactate levels, inflammation, and mitochondrial function in experimental sepsis","volume":"21","author":"Correa","year":"2017","journal-title":"Crit. Care"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0080","doi-asserted-by":"crossref","first-page":"16082","DOI":"10.1038\/s41598-017-16463-6","article-title":"Increased inflammation, oxidative stress and mitochondrial respiration in brown adipose tissue from obese mice","volume":"7","author":"Alcala","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0085","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1111\/cei.12899","article-title":"Systemic inflammation in acute intermittent porphyria: a case-control study","volume":"187","author":"Storjord","year":"2017","journal-title":"Clin. Exp. Immunol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0090","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1001\/jamadermatol.2017.1557","article-title":"Clinical, biochemical, and genetic characterization of north American patients with Erythropoietic Protoporphyria and X-linked Protoporphyria","volume":"153","author":"Balwani","year":"2017","journal-title":"JAMA Dermatol"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0095","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1016\/j.cgh.2012.08.038","article-title":"Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda","volume":"10","author":"Singal","year":"2012","journal-title":"Clin. Gastroenterol. Hepatol.: Offl. Clin. Pract. J. Am. Gastroenterol. Assoc."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0100","doi-asserted-by":"crossref","first-page":"E585","DOI":"10.1152\/ajpendo.00093.2013","article-title":"Dysfunctional mitochondrial bioenergetics and oxidative stress in Akita(+\/Ins2)-derived beta-cells","volume":"305","author":"Mitchell","year":"2013","journal-title":"Am. J Physiol. Endocrinol. Metab."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0105","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.trsl.2010.04.001","article-title":"Altered mitochondrial membrane potential, mass, and morphology in the mononuclear cells of humans with type 2 diabetes","volume":"156","author":"Widlansky","year":"2010","journal-title":"Trans. Res.: J. Lab. Clin. Med."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0110","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1097\/CCM.0b013e31820eda5c","article-title":"Bioenergetic failure of human peripheral blood monocytes in patients with septic shock is mediated by reduced F1Fo adenosine-5\u2032-triphosphate synthase activity","volume":"39","author":"Japiassu","year":"2011","journal-title":"Crit. Care Med."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0115","doi-asserted-by":"crossref","first-page":"709061","DOI":"10.4061\/2011\/709061","article-title":"May \"mitochondrial eve\" and mitochondrial haplogroups play a role in neurodegeneration and Alzheimer's disease?","volume":"2011","author":"Ienco","year":"2011","journal-title":"Int. J. Alzheimers Dis."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0120","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1002\/hep.24377","article-title":"Mitochondria-targeted ubiquinone (MitoQ) decreases ethanol-dependent micro and macro hepatosteatosis","volume":"54","author":"Chacko","year":"2011","journal-title":"Hepatology"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0125","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1042\/CS20140101","article-title":"The Bioenergetic Health Index: a new concept in mitochondrial translational research","volume":"127","author":"Chacko","year":"2014","journal-title":"Clin. Sci."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0130","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1038\/labinvest.2013.53","article-title":"Methods for defining distinct bioenergetic profiles in platelets, lymphocytes, monocytes, and neutrophils, and the oxidative burst from human blood","volume":"93","author":"Chacko","year":"2013","journal-title":"Lab. Invest. J Tech. Method Pathol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0140","article-title":"Bioenergetics and the oxidative burst: protocols for the isolation and evaluation of human leukocytes and platelets","volume":"85","author":"Kramer","year":"2014","journal-title":"J. Vis. Exp."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0150","doi-asserted-by":"crossref","first-page":"1621","DOI":"10.1016\/j.freeradbiomed.2011.08.005","article-title":"Assessing bioenergetic function in response to oxidative stress by metabolic profiling","volume":"51","author":"Dranka","year":"2011","journal-title":"Free Rad. Biol. Med."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0155","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/j.redox.2013.12.026","article-title":"A review of the mitochondrial and glycolytic metabolism in human platelets and leukocytes: implications for their use as bioenergetic biomarkers","volume":"2","author":"Kramer","year":"2014","journal-title":"Redox Biol."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0160","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1042\/BJ20090934","article-title":"Importance of the bioenergetic reserve capacity in response to cardiomyocyte stress induced by 4-hydroxynonenal","volume":"424","author":"Hill","year":"2009","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0165","doi-asserted-by":"crossref","first-page":"2525","DOI":"10.1016\/j.bbadis.2015.08.026","article-title":"Defining the effects of storage on platelet bioenergetics: the role of increased proton leak","volume":"1852","author":"Ravi","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0170","doi-asserted-by":"crossref","first-page":"26424","DOI":"10.1074\/jbc.M110.215772","article-title":"Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells","volume":"286","author":"Tian","year":"2011","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0175","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1038\/nature07201","article-title":"Origin and physiological roles of inflammation","volume":"454","author":"Medzhitov","year":"2008","journal-title":"Nature"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0180","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1038\/nature08780","article-title":"Circulating mitochondrial DAMPs cause inflammatory responses to injury","volume":"464","author":"Zhang","year":"2010","journal-title":"Nature"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0185","series-title":"Porphyria Cutanea Tarda","author":"Singal","year":"2014"},{"key":"10.1016\/j.ymgmr.2019.100451_bb0190","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1055\/s-2008-1040704","article-title":"Protoporphyria","volume":"2","author":"Bloomer","year":"1982","journal-title":"Semin. Liver Dis."},{"key":"10.1016\/j.ymgmr.2019.100451_bb0195","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1007\/BF00509083","article-title":"Photodynamic action of uroporphyrin on the complement system in porphyria cutanea tarda","volume":"277","author":"Meurer","year":"1985","journal-title":"Arch. Dermatol. Res."}],"container-title":["Molecular Genetics and Metabolism Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2214426918301472?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2214426918301472?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T12:51:06Z","timestamp":1762001466000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2214426918301472"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6]]},"references-count":37,"alternative-id":["S2214426918301472"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgmr.2019.100451","relation":{},"ISSN":["2214-4269"],"issn-type":[{"value":"2214-4269","type":"print"}],"subject":[],"published":{"date-parts":[[2019,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Feasibility of cellular bioenergetics as a biomarker in porphyria patients","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgmr.2019.100451","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"100451"}}