دورية أكاديمية
Lipid and energy metabolism in Wilson disease.
| العنوان: | Lipid and energy metabolism in Wilson disease. |
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| المؤلفون: | Mazi TA; Department of Nutrition, University of California Davis, Davis, CA, USA.; Department of Community Health Sciences, College of Applied Medical Sciences, King Saud University, Riyadh, Saudi Arabia., Shibata NM; Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of California Davis, Sacramento, CA, USA., Medici V; Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of California Davis, Sacramento, CA, USA. |
| المصدر: | Liver research [Liver Res] 2020 Mar; Vol. 4 (1), pp. 5-14. Date of Electronic Publication: 2020 Feb 19. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Ke Ai Country of Publication: China NLM ID: 101705555 Publication Model: Print-Electronic Cited Medium: Print ISSN: 2096-2878 (Print) NLM ISO Abbreviation: Liver Res Subsets: PubMed not MEDLINE |
| أسماء مطبوعة: | Original Publication: Beijing : Ke Ai |
| مستخلص: | Copper accumulation and deficiency are reciprocally connected to lipid metabolism. In Wilson disease (WD), which is caused by a genetic loss of function of the copper-transporting P-type ATPase beta, copper accumulates mainly in the liver and lipid metabolism is dysregulated. The underlying mechanisms linking copper and lipid metabolism in WD are not clear. Copper may impair metabolic machinery by direct binding to protein and lipid structures or by generating reactive oxygen species with consequent damage to cellular organelles vital to energy metabolism. In the liver, copper overload results in mitochondrial impairment, down-regulation of lipid metabolism, and the development of steatosis with an etiology not fully elucidated. Little is known regarding the effect of copper overload on extrahepatic energy homeostasis. This review aims to discuss alterations in hepatic energy metabolism associated with WD, highlights potential mechanisms involved in the development of hepatic and systemic dysregulation of lipid metabolism, and reviews current knowledge on the effects of copper overload on extrahepatic energy metabolism. Competing Interests: Declaration of competing interest The authors declare they have no conflict of interest. |
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| معلومات مُعتمدة: | R01 DK104770 United States DK NIDDK NIH HHS |
| فهرسة مساهمة: | Keywords: (ATP7B); Carbohydrate; Copper; Copper-transporting P-type ATPase B; Lipid; Metabolism; Steatosis |
| تواريخ الأحداث: | Date Created: 20200825 Latest Revision: 20200928 |
| رمز التحديث: | 20221213 |
| مُعرف محوري في PubMed: | PMC7437987 |
| DOI: | 10.1016/j.livres.2020.02.002 |
| PMID: | 32832193 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 2096-2878 |
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| DOI: | 10.1016/j.livres.2020.02.002 |