دورية أكاديمية
أعرض في EDS

Dietary Supplementation with n-3 Polyunsaturated Fatty Acids Delays the Phenotypic Manifestation of Krabbe Disease and Partially Restores Lipid Mediator Production in the Brain-Study in a Mouse Model of the Disease.

التفاصيل البيبلوغرافية
العنوان: Dietary Supplementation with n-3 Polyunsaturated Fatty Acids Delays the Phenotypic Manifestation of Krabbe Disease and Partially Restores Lipid Mediator Production in the Brain-Study in a Mouse Model of the Disease.
المؤلفون: Signorini C; Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy., Pannuzzo G; Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy., Graziano ACE; Department of Medicine and Surgery, University of Enna 'Kore', 94100 Enna, Italy., Moretti E; Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy., Collodel G; Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy., Cardile V; Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy.
المصدر: International journal of molecular sciences [Int J Mol Sci] 2024 Jun 28; Vol. 25 (13). Date of Electronic Publication: 2024 Jun 28.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: MDPI Country of Publication: Switzerland NLM ID: 101092791 Publication Model: Electronic Cited Medium: Internet ISSN: 1422-0067 (Electronic) Linking ISSN: 14220067 NLM ISO Abbreviation: Int J Mol Sci Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Basel, Switzerland : MDPI, [2000-
مواضيع طبية MeSH: Dietary Supplements* , Fatty Acids, Omega-3*/metabolism , Fatty Acids, Omega-3*/pharmacology , Fatty Acids, Omega-3*/administration & dosage , Disease Models, Animal* , Brain*/metabolism , Brain*/drug effects , Leukodystrophy, Globoid Cell*/diet therapy , Leukodystrophy, Globoid Cell*/metabolism , Leukodystrophy, Globoid Cell*/drug therapy , Leukodystrophy, Globoid Cell*/genetics, Animals ; Mice ; Phenotype ; Docosahexaenoic Acids/pharmacology ; Docosahexaenoic Acids/metabolism ; Lipid Metabolism/drug effects ; Dinoprost/analogs & derivatives ; Dinoprost/metabolism ; Male
مستخلص: Lipid mediators from fatty acid oxidation have been shown to be associated with the severity of Krabbe disease (KD), a disorder linked to mutations in the galactosylceramidase ( GALC ) gene. This study aims to investigate the effects of n-3 polyunsaturated fatty acid (PUFA) supplementation on KD traits and fatty acid metabolism using Twitcher (Tw) animals as a natural model for KD. Wild-type (Wt), heterozygous (Ht), and affected Tw animals were treated orally with 36 mg n-3 PUFAs/kg body weight/day from 10 to 35 days of life. The end product of PUFA peroxidation (8-isoprostane), the lipid mediator involved in the resolution of inflammatory exudates (resolvin D1), and the total amount of n-3 PUFAs were analyzed in the brains of mice. In Tw mice, supplementation with n-3 PUFAs delayed the manifestation of disease symptoms ( p < 0.0001), and in the bran, decreased 8-isoprostane amounts ( p < 0.0001), increased resolvin D1 levels ( p < 0.005) and increased quantity of total n-3 PUFAs ( p < 0.05). Furthermore, total brain n-3 PUFA levels were associated with disease severity (r = -0.562, p = 0.0001), resolvin D1 (r = 0.712, p < 0.0001), and 8-isoprostane brain levels (r = -0.690, p < 0.0001). For the first time in a natural model of KD, brain levels of n-3 PUFAs are shown to determine disease severity and to be involved in the peroxidation of brain PUFAs as well as in the production of pro-resolving lipid mediators. It is also shown that dietary supplementation with n-3 PUFAs leads to a slowing of the phenotypic presentation of the disease and restoration of lipid mediator production.
References: Front Psychiatry. 2020 Feb 28;11:122. (PMID: 32180741)
J Alzheimers Dis. 2015;48(2):293-301. (PMID: 26401996)
Nat Rev Drug Discov. 2016 Aug;15(8):551-67. (PMID: 27020098)
PLoS One. 2017 May 22;12(5):e0178103. (PMID: 28531236)
Molecules. 2023 Aug 22;28(17):. (PMID: 37687017)
Front Neurol. 2023 Mar 09;14:1130439. (PMID: 36970527)
J Nutr. 2007 Apr;137(4):855-9. (PMID: 17374644)
Biochim Biophys Acta. 1994 Jan 3;1210(2):244-8. (PMID: 8280777)
J Lipid Res. 2009 Apr;50 Suppl:S219-23. (PMID: 18957694)
Front Pharmacol. 2021 Feb 03;11:610734. (PMID: 33732145)
Nutrients. 2021 Jul 15;13(7):. (PMID: 34371930)
Mol Nutr Food Res. 2022 Nov;66(22):e2200275. (PMID: 36099650)
Immunotargets Ther. 2023 Oct 31;12:105-111. (PMID: 37928748)
Mediators Inflamm. 2020 Aug 31;2020:3640450. (PMID: 32934603)
Brain Sci. 2023 Sep 02;13(9):. (PMID: 37759879)
Int J Mol Sci. 2010 Nov 17;11(11):4631-59. (PMID: 21151461)
J Neurosci. 2020 Apr 8;40(15):3104-3118. (PMID: 32127495)
Mar Drugs. 2023 Dec 27;22(1):. (PMID: 38248642)
Free Radic Biol Med. 2019 Aug 1;139:46-54. (PMID: 31100476)
Int J Mol Sci. 2023 May 30;24(11):. (PMID: 37298468)
Neuropsychopharmacology. 2018 Feb;43(3):534-545. (PMID: 28741625)
Brain Res Bull. 2023 Jul;199:110660. (PMID: 37149267)
Nutrition. 2024 Jul;123:112413. (PMID: 38518540)
Eur J Nutr. 2024 Jun;63(4):1003-1022. (PMID: 38300291)
Gene Ther. 2004 Aug;11(15):1188-94. (PMID: 15164096)
Altern Med Rev. 2007 Sep;12(3):207-27. (PMID: 18072818)
Mar Drugs. 2024 Feb 06;22(2):. (PMID: 38393051)
FASEB J. 2004 Dec;18(15):1791-800. (PMID: 15576482)
J Neurotrauma. 2020 Jan 1;37(1):66-79. (PMID: 31256709)
Biomolecules. 2023 Oct 23;13(10):. (PMID: 37892244)
Mol Neurobiol. 2018 Aug;55(8):6894-6905. (PMID: 29357041)
Pharmacol Ther. 2014 Mar;141(3):272-82. (PMID: 24201219)
Sci Rep. 2024 Feb 5;14(1):2977. (PMID: 38316895)
JAMA Pediatr. 2024 Jan 1;178(1):45-54. (PMID: 37983037)
Talanta. 2018 Jul 1;184:193-201. (PMID: 29674032)
Stem Cells. 2011 Jan;29(1):67-77. (PMID: 21280158)
Nutrients. 2018 Apr 29;10(5):. (PMID: 29710835)
J Clin Neurosci. 2021 Dec;94:237-243. (PMID: 34863444)
Curr Opin Lipidol. 2016 Feb;27(1):54-66. (PMID: 26709472)
Front Immunol. 2021 Jul 12;12:714042. (PMID: 34322137)
Prostaglandins Leukot Essent Fatty Acids. 2014 Nov;91(5):183-93. (PMID: 25240461)
Front Mol Neurosci. 2022 May 10;15:896314. (PMID: 35620447)
PLoS One. 2013 Jun 19;8(6):e66418. (PMID: 23840462)
Nutrients. 2016 Jan 04;8(1):. (PMID: 26742060)
Mol Ther. 2007 Jan;15(1):44-52. (PMID: 17164774)
J Alzheimers Dis. 2018;62(3):1013-1022. (PMID: 29103035)
Cells. 2023 Feb 09;12(4):. (PMID: 36831228)
Nat Commun. 2020 Nov 30;11(1):6133. (PMID: 33257673)
Cell Rep. 2023 Jun 27;42(6):112617. (PMID: 37285269)
J Nutr Biochem. 2019 Jan;63:35-43. (PMID: 30321750)
Genes Nutr. 2012 Jul;7(3):447-58. (PMID: 22399313)
Mar Drugs. 2023 Jun 09;21(6):. (PMID: 37367679)
Mar Drugs. 2023 Jul 10;21(7):. (PMID: 37504929)
J Neurosci Res. 2016 Nov;94(11):1341-8. (PMID: 27638616)
Lipids. 2014 Nov;49(11):1115-25. (PMID: 25208498)
Front Physiol. 2021 Mar 30;12:645646. (PMID: 33868013)
Nutrients. 2023 Jul 14;15(14):. (PMID: 37513559)
Clin Nutr. 2024 Jan;43(1):176-186. (PMID: 38061271)
Brain Behav Immun. 2024 Feb;116:385-401. (PMID: 38145855)
معلومات مُعتمدة: 20821141006/55048726 Partially funded by Italian Association for Leucodistrophy of Krabbe "Progetto Grazia"
فهرسة مساهمة: Keywords: Krabbe disease; brain; fatty acid profile; isoprostanes; omega-3 PUFA; omega-3 enriched diet; resolvins
المشرفين على المادة: 0 (Fatty Acids, Omega-3)
25167-62-8 (Docosahexaenoic Acids)
B7IN85G1HY (Dinoprost)
0 (resolvin D1)
27415-26-5 (8-epi-prostaglandin F2alpha)
تواريخ الأحداث: Date Created: 20240713 Date Completed: 20240713 Latest Revision: 20240715
رمز التحديث: 20240715
مُعرف محوري في PubMed: PMC11241235
DOI: 10.3390/ijms25137149
PMID: 39000257
قاعدة البيانات: MEDLINE
الوصف
تدمد:1422-0067
DOI:10.3390/ijms25137149