| Fatty acid 2-Hydroxylation in mammalian sphingolipid biology. | |
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MedLine Citation:
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PMID: 20026285 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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2-Hydroxy fatty acids (hFA) are important components of a subset of mammalian sphingolipids. The presence of hFA in sphingolipids is best described in the nervous system, epidermis, and kidney. However, the literature also indicates that various hFA-sphingolipids are present in additional tissues and cell types, as well as in tumors. Biosynthesis of hFA-sphingolipids requires fatty acid 2-hydroyxlase, and degradation of hFA-sphingolipids depends, at least in part, on lysosomal acid ceramidase and the peroxisomal fatty acid alpha-oxidation pathway. Mutations in the fatty acid 2-hydroxylase gene, FA2H, have been associated with leukodystrophy and spastic paraparesis in humans, underscoring the importance of hFA-sphingolipids in the nervous system. In the epidermis, hFA-ceramides are essential for the permeability barrier function. Physiological function of hFA-sphingolipids in other organs remains largely unknown. Recent evidence indicates that hFA-sphingolipids have specific roles in cell signaling. |
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Authors:
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Hiroko Hama |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Review Date: 2009-12-21 |
Journal Detail:
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Title: Biochimica et biophysica acta Volume: 1801 ISSN: 0006-3002 ISO Abbreviation: Biochim. Biophys. Acta Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-02-22 Completed Date: 2010-04-20 Revised Date: 2011-09-26 |
Medline Journal Info:
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Nlm Unique ID: 0217513 Medline TA: Biochim Biophys Acta Country: Netherlands |
Other Details:
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Languages: eng Pagination: 405-14 Citation Subset: IM |
Copyright Information:
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2009 Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Biochemistry and Molecular Biology, Medical University of South Carolina, Charleston, SC 29425, USA. hama@musc.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Fatty Acids / metabolism* Humans Hydroxylation Mixed Function Oxygenases / metabolism* Sphingolipids / metabolism* |
| Grant Support | |
ID/Acronym/Agency:
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NS056075/NS/NINDS NIH HHS; NS060807/NS/NINDS NIH HHS; R01 NS060807-01A1/NS/NINDS NIH HHS; R01 NS060807-02/NS/NINDS NIH HHS; R03 NS056075-01/NS/NINDS NIH HHS; R03 NS056075-02/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids; 0/Sphingolipids; EC 1.-/Mixed Function Oxygenases; EC 1.-/fatty acid alpha-hydroxylase |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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