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As it leads to the death of neurons in the cerebral cortex, the patients primarily present with neurological symptoms, but recently metabolic changes resulting from mitochondrial dysfunction have been identified as novel pathological features. The carnitine shuttle is a complex consisting of three enzymes whose function is to transport the long-chain fatty acids into the mitochondria. Here, its pharmacological modification was used to test the hypothesis that shifting metabolism to lipid oxidation exacerbates the HD symptoms. Behavioural and transcriptional analyses were carried out on HD Drosophila model, to evaluate the involvement of the carnitine cycle in this pathogenesis. Pharmacological inhibition of CPT1, the rate-limiting enzyme of the carnitine cycle, ameliorates the HD symptoms in Drosophila, likely acting on the expression of carnitine-related genes.<\/jats:p>","DOI":"10.3390\/molecules27103125","type":"journal-article","created":{"date-parts":[[2022,5,13]],"date-time":"2022-05-13T08:37:02Z","timestamp":1652431022000},"page":"3125","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The Reversible Carnitine Palmitoyltransferase 1 Inhibitor (Teglicar) Ameliorates the Neurodegenerative Phenotype in a Drosophila Huntington\u2019s Disease Model by Acting on the Expression of Carnitine-Related Genes"],"prefix":"10.3390","volume":"27","author":[{"given":"Carla","family":"Bertapelle","sequence":"first","affiliation":[{"name":"Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy"},{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Rosaria","family":"Carillo","sequence":"additional","affiliation":[{"name":"Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy"},{"name":"Department of Experimental Medicine, University of Campania \u201cLuigi Vanvitelli\u201d, Via Santa Maria di Costantinopoli 16, 80138 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0780-9844","authenticated-orcid":false,"given":"Nunzio Antonio","family":"Cacciola","sequence":"additional","affiliation":[{"name":"Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy"},{"name":"Department of Veterinary Medicine and Animal Productions, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3643-9889","authenticated-orcid":false,"given":"Yulii V.","family":"Shidlovskii","sequence":"additional","affiliation":[{"name":"Department of Gene Expression Regulation in Development, Institute of Gene Biology, Russian Academy of Sciences, 34\/5 Vavilov St., 119334 Moscow, Russia"},{"name":"Department of Biology and General Genetics, I.M. Sechenov First Moscow State Medical University, 8, bldg. 2 Trubetskaya St., 119048 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianfranco","family":"Peluso","sequence":"additional","affiliation":[{"name":"Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9342-3760","authenticated-orcid":false,"given":"Filomena Anna","family":"Digilio","sequence":"additional","affiliation":[{"name":"Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/s41582-020-0389-4","article-title":"Huntington disease: New insights into molecular pathogenesis and therapeutic opportunities","volume":"16","author":"Tabrizi","year":"2020","journal-title":"Nat. Rev. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"38","DOI":"10.2174\/138920291501140306113344","article-title":"The Mechanistic Links Between Proteasome Activity, Aging and Agerelated Diseases","volume":"15","author":"Saez","year":"2014","journal-title":"Curr. Genom."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Koyuncu, S., Fatima, A., Gutierrez-Garcia, R., and Vilchez, D. (2017). Proteostasis of huntingtin in health and disease. Int. J. Mol. Sci., 18.","DOI":"10.3390\/ijms18071568"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1016\/j.molcel.2016.08.032","article-title":"Resetting Epigenetic Memory by Reprogramming of Histone Modifications in Mammals","volume":"63","author":"Zheng","year":"2016","journal-title":"Mol. Cell"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.bbadis.2009.07.012","article-title":"Mitochondria in Huntington\u2019s disease","volume":"1802","author":"Damiano","year":"2010","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1016\/j.neuron.2016.02.003","article-title":"Review The Biology of Huntingtin","volume":"89","author":"Saudou","year":"2016","journal-title":"Neuron"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","article-title":"Huntington\u2019s disease: From molecular pathogenesis to clinical treatment","volume":"10","author":"Ross","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1002\/ajmg.a.38494","article-title":"Therapy development in Huntington disease: From current strategies to emerging opportunities","volume":"176","author":"Dickey","year":"2019","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"65","DOI":"10.3233\/JHD-150181","article-title":"Partial Amelioration of Peripheral and Central Symptoms of Huntington\u2019s Disease via Modulation of Lipid Metabolism","volume":"5","author":"Chen","year":"2016","journal-title":"J. Huntingt. Dis."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jacl.2009.11.003","article-title":"Altered cholesterol and fatty acid metabolism in Huntington disease","volume":"4","author":"Block","year":"2011","journal-title":"J. Clin. Lipidol."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Nambron, R., Silajd, E., Kalliolia, E., Ottolenghi, C., Hindmarsh, P., Hill, N.R., Costelloe, S.J., Martin, N.G., Positano, V., and Watt, H.C. (2016). A Metabolic Study of Huntington\u2019s Disease. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0146480"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1084","DOI":"10.1016\/j.bbrc.2016.09.065","article-title":"Energy defects in Huntington\u2019s disease: Why \u201cin vivo\u201d evidence matters","volume":"483","author":"Liot","year":"2017","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1172\/JCI45691","article-title":"Energy deficit in Huntington disease: Why it matters","volume":"121","author":"Mochel","year":"2011","journal-title":"J. Clin. Investig."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"9233","DOI":"10.1002\/jcp.27602","article-title":"Meldonium improves Huntington\u2019s disease mitochondrial dysfunction by restoring peroxisome proliferator-activated receptor \u03b3 coactivator 1\u03b1 expression","volume":"234","author":"Finicelli","year":"2019","journal-title":"J. Cell. Physiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1007\/s11010-015-2561-5","article-title":"Mitochondrial modulators improve lipid composition and attenuate memory deficits in experimental model of Huntington\u2019s disease","volume":"410","author":"Mehrotra","year":"2015","journal-title":"Mol. Cell. Biochem."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1111\/nan.12286","article-title":"Evidence for altered cholesterol metabolism in Huntington\u2019s disease post mortem brain tissue","volume":"42","author":"Kreilaus","year":"2016","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1007\/s11010-014-2098-z","article-title":"Carnitine\/acylcarnitine translocase and carnitine palmitoyltransferase 2 form a complex in the inner mitochondrial membrane","volume":"394","author":"Console","year":"2014","journal-title":"Mol. Cell. Biochem."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Carillo, M.R., Bertapelle, C., Scial\u00f2, F., Siervo, M., Spagnuolo, G., Simeone, M., Peluso, G., and Digilio, F.A. (2020). L-Carnitine in Drosophila: A Review. Antioxidants, 9.","DOI":"10.3390\/antiox9121310"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2422","DOI":"10.1016\/j.bbamcr.2016.01.023","article-title":"Carnitine transport and fatty acid oxidation hhs public access author manuscript","volume":"1863","author":"Longo","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.pcl.2017.11.006","article-title":"Inborn Errors of Metabolism with Myopathy: Defects of Fatty Acid Oxidation and the Carnitine Shuttle System","volume":"65","author":"Vockley","year":"2018","journal-title":"Pediatric Clin. N. Am."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1093\/jnci\/djt030","article-title":"Carnitine-acyltransferase system inhibition, cancer cell death, and prevention of myc-induced lymphomagenesis","volume":"105","author":"Pacilli","year":"2013","journal-title":"J. Natl. Cancer Inst."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1925","DOI":"10.1182\/blood-2014-12-617498","article-title":"Targeting the leukemia cell metabolism by the CPT1a inhibition: Functional preclinical effects in leukemias","volume":"126","author":"Ricciardi","year":"2015","journal-title":"Blood"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1745","DOI":"10.1042\/CS20190587","article-title":"Fatty acid oxidation inhibitor etomoxir suppresses tumor progression and induces cell cycle arrest via PPAR\u03b3-mediated pathway in bladder cancer","volume":"133","author":"Cheng","year":"2019","journal-title":"Clin. Sci."},{"key":"ref_24","first-page":"238","article-title":"Carnitine Palmitoyltransferase Inhibitor in Diabetes","volume":"10","author":"Chen","year":"2016","journal-title":"J. Mol. Genet. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/j.biopha.2009.06.020","article-title":"Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington\u2019s disease","volume":"64","author":"Vamos","year":"2010","journal-title":"Biomed. Pharmacother."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1007\/s00401-010-0788-5","article-title":"Modulation of lipid peroxidation and mitochondrial function improves neuropathology in Huntington\u2019s disease mice","volume":"121","author":"Lee","year":"2011","journal-title":"Acta Neuropathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"267","DOI":"10.3233\/JHD-170264","article-title":"Towards an Understanding of Energy Impairment in Huntington\u2019s Disease Brain","volume":"6","author":"Dubinsky","year":"2017","journal-title":"J. Huntingt. Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1101\/gr.169101","article-title":"A systematic analysis of human disease-associated gene sequences in Drosophila melanogaster","volume":"11","author":"Reiter","year":"2001","journal-title":"Genome Res."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1534\/genetics.115.179457","article-title":"Drosophila as an In Vivo Model for Human Neurodegenerative Disease","volume":"201","author":"Mcgurk","year":"2015","journal-title":"Genetics"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"31411","DOI":"10.1038\/srep31411","article-title":"Altered lipid metabolism in Drosophila model of Huntington\u2019s disease","volume":"6","author":"Aditi","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"166127","DOI":"10.1016\/j.bbadis.2021.166127","article-title":"Deciphering the key mechanisms leading to alteration of lipid metabolism in Drosophila model of Huntington\u2019s disease","volume":"1867","author":"Singh","year":"2021","journal-title":"Biochim. Biophys. Acta (BBA) Mol. Basis Dis."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1139\/G08-023","article-title":"Mutations of the withered (whd) gene in Drosophila melanogaster confer hypersensitivity to oxidative stress and are lesions of the carnitine palmitoyltransferase I (CPT I) gene","volume":"51","author":"Strub","year":"2008","journal-title":"Genome"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"7805","DOI":"10.1038\/srep07805","article-title":"Glial \u03b2-Oxidation regulates drosophila energy metabolism","volume":"5","author":"Schulz","year":"2015","journal-title":"Sci. Rep."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.ymgme.2005.02.012","article-title":"dif-1 and colt, both implicated in early embryonic development, encode carnitine acylcarnitine translocase","volume":"85","author":"Oey","year":"2005","journal-title":"Mol. Genet. Metab."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Engelhart, D.C., Azad, P., Ali, S., Granados, J.C., Haddad, G.G., and Nigam, S.K. (2020). Drosophila SLC22 orthologs related to OATs, OCTs, and OCTNs regulate development and responsiveness to oxidative stress. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21062002"},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"Laranjeira, A., Schulz, J., and Dotti, C.G. (2016). Genes related to fatty acid \u03b2-oxidation play a role in the functional decline of the drosophila brain with age. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0161143"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.cmet.2009.01.009","article-title":"Drosophila HNF4 Regulates Lipid Mobilization and \u03b2-Oxidation","volume":"9","author":"Palanker","year":"2009","journal-title":"Cell Metab."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1534\/genetics.118.301583","article-title":"Triacylglycerol metabolism in drosophila melanogaster","volume":"210","author":"Heier","year":"2018","journal-title":"Genetics"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"2039","DOI":"10.1096\/fj.12-216689","article-title":"Carnitine palmitoyltransferase 2 and carnitine\/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines","volume":"27","author":"Violante","year":"2013","journal-title":"FASEB J."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1146\/annurev.physiol.64.082201.154705","article-title":"Fatty acid oxidation disorders","volume":"64","author":"Rinaldo","year":"2002","journal-title":"Annu. Rev. Physiol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1535","DOI":"10.1534\/genetics.116.195776","article-title":"Pervasive Behavioral Effects of MicroRNA Regulation in Drosophila","volume":"206","author":"Patraquim","year":"2017","journal-title":"Genetics"},{"key":"ref_42","doi-asserted-by":"crossref","unstructured":"Mishra, M. (2020). Fundamental Approaches to Screen Abnormalities in Drosophila (Springer Protocols Handbooks), Springer. [1st ed.].","DOI":"10.1007\/978-1-4939-9756-5"},{"key":"ref_43","first-page":"721","article-title":"Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis","volume":"733","author":"Estes","year":"2013","journal-title":"Dis. Models Mech."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Di Cristo, F., Calarco, A., Digilio, F.A., Sinicropi, M.S., Rosano, C., Galderisi, U., Melone, M.A.B., Saturnino, C., and Peluso, G. (2020). The discovery of highly potent thp derivatives as octn2 inhibitors: From structure-based virtual screening to in vivo biological activity. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197431"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"32492","DOI":"10.1074\/jbc.M805832200","article-title":"Overexpression of glucose-6-phosphate dehydrogenase extends the life span of Drosophila melanogaster","volume":"283","author":"Legan","year":"2008","journal-title":"J. Biol. Chem."},{"key":"ref_46","doi-asserted-by":"crossref","unstructured":"Besson, M.T., Alegr\u00eda, K., Garrido-Gerter, P., Barros, L.F., and Li\u00e9vens, J.C. (2015). Enhanced neuronal glucose transporter expression reveals metabolic choice in a HD Drosophila model. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0118765"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1023\/B:NERE.0000014824.04728.dd","article-title":"The Energetics of Huntington\u2019s Disease","volume":"29","author":"Browne","year":"2004","journal-title":"Neurochem. Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"182","DOI":"10.3389\/fnmol.2018.00182","article-title":"Medium-chain fatty acids, beta-hydroxybutyric acid and genetic modulation of the carnitine shuttle are protective in a drosophila model of ALS based on TDP-43","volume":"11","author":"Manzo","year":"2018","journal-title":"Front. Mol. Neurosci."},{"key":"ref_49","first-page":"4764","article-title":"Human single-chain Fv intrabodies counteract in situ huntingtin aggregation in cellular models of Huntington\u2019s disease","volume":"98","author":"Wolfgang","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Martin, E., Heidari, R., Monnier, V., and Tricoire, H. (2021). Genetic screen in adult drosophila reveals that dCBP depletion in glial cells mitigates huntington disease pathology through a foxo-dependent pathway. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22083884"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/978-1-4939-7825-0_5","article-title":"Nonmammalian models of Huntington\u2019s disease","volume":"1780","author":"Chongtham","year":"2018","journal-title":"Methods Mol. Biol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"15583","DOI":"10.1038\/s41598-020-72638-8","article-title":"Dysregulation of metabolic pathways by carnitine palmitoyl-transferase 1 plays a key role in central nervous system disorders: Experimental evidence based on animal models","volume":"10","author":"Trabjerg","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"M\u00f8rkholt, A.S., Oklinski, M.K., Larsen, A., Bockermann, I.D.R., Issazadeh-Navikas, S., Goller, J., Nieland, K., Kwon, T.-H., Corthals Id, A., and Nielsen, S. (2020). Pharmacological inhibition of carnitine palmitoyl transferase 1 inhibits and reverses experimental autoimmune encephalitis in rodents. PLoS ONE, 15.","DOI":"10.1371\/journal.pone.0234493"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"2158","DOI":"10.1038\/s41598-017-02343-6","article-title":"Blocking of carnitine palmitoyl transferase 1 potently reduces stress-induced depression in rat highlighting a pivotal role of lipid metabolism","volume":"7","author":"Wiborg","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1002\/(SICI)1097-4547(19980701)53:1<66::AID-JNR7>3.0.CO;2-H","article-title":"Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS","volume":"53","author":"Hall","year":"1998","journal-title":"J. Neurosci. Res."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1111\/j.1440-1789.2006.00662.x","article-title":"Neurofibrillary tangles and deposition of oxidative products in the brain in cases of myotonic dystrophy","volume":"26","author":"Oyamada","year":"2006","journal-title":"Neuropathology"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1080\/01616412.2016.1251711","article-title":"Oxidative stress and mitochondrial dysfunction-linked neurodegenerative disorders","volume":"39","author":"Islam","year":"2017","journal-title":"Neurol. Res."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1218","DOI":"10.2174\/1389450111007011218","article-title":"Mitochondrial-Associated Metabolic Changes and Neurodegeneration in Huntingtons Disease\u2014From Clinical Features to the Bench","volume":"11","author":"Rosenstock","year":"2012","journal-title":"Curr. Drug Targets"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1152\/physrev.00025.2005","article-title":"Transcriptional regulation of metabolism","volume":"86","author":"Desvergne","year":"2006","journal-title":"Physiol. Rev."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2345","DOI":"10.1101\/gad.289140.116","article-title":"Undercover: Gene control by metabolites and metabolic enzymes","volume":"30","author":"Verrijzer","year":"2016","journal-title":"Genes Dev."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1464","DOI":"10.1016\/B0-12-227055-X\/00263-7","article-title":"Coconut Palm","volume":"1","author":"Lal","year":"2003","journal-title":"Encycl. Food Sci. Nutr."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Bravo-Ruiz, I., Medina, M.\u00c1., and Mart\u00ednez-Poveda, B. (2021). From food to genes: Transcriptional regulation of metabolism by lipids and carbohydrates. Nutrients, 13.","DOI":"10.3390\/nu13051513"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1242\/dev.118.2.401","article-title":"Targeted gene expression as a means of altering cell fates and generating dominant phenotypes","volume":"118","author":"Brand","year":"1993","journal-title":"Development"},{"key":"ref_64","first-page":"e59535","article-title":"Methods to test endocrine disruption in drosophila melanogaster","volume":"2019","author":"Bovier","year":"2019","journal-title":"J. Vis. Exp."}],"container-title":["Molecules"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1420-3049\/27\/10\/3125\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:10:21Z","timestamp":1760137821000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1420-3049\/27\/10\/3125"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,13]]},"references-count":64,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2022,5]]}},"alternative-id":["molecules27103125"],"URL":"https:\/\/doi.org\/10.3390\/molecules27103125","relation":{},"ISSN":["1420-3049"],"issn-type":[{"value":"1420-3049","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,5,13]]}}}