دورية أكاديمية
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.
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. |
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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. |
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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 |
تدمد: | 1422-0067 |
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DOI: | 10.3390/ijms25137149 |