کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1904918 1534680 2012 11 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Functions of plasmalogen lipids in health and disease
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
پیش نمایش صفحه اول مقاله
Functions of plasmalogen lipids in health and disease
چکیده انگلیسی

Plasmalogens are a unique class of membrane glycerophospholipids containing a fatty alcohol with a vinyl-ether bond at the sn-1 position, and enriched in polyunsaturated fatty acids at the sn-2 position of the glycerol backbone. These two features provide novel properties to these compounds. Although plasmalogens represent up to 20% of the total phospholipid mass in humans their physiological roles have been challenging to identify, and are likely to be particular to different tissues, metabolic processes and developmental stages. Their biosynthesis starts in peroxisomes, and defects at these steps cause the malformation syndrome, Rhizomelic Chondrodysplasia Punctata (RCDP). The RCDP phenotype predicts developmental roles for plasmalogens in bone, brain, lens, lung, kidney and heart. Recent studies have revealed secondary plasmalogen deficiencies associated with more common disorders and allow us to tease out additional pathways dependent on plasmalogen functions. In this review, we present current knowledge of plasmalogen biology in health and disease. This article is part of a Special Issue entitled: Metabolic Functions and Biogenesis of peroxisomes in Health and Disease.


► Plasmalogen synthesis and regulation.
► Unique roles ascribed to these molecules: anti-oxidants, membrane structure, signal transduction.
► Primary plasmalogen deficiency disease states: RCDP.
► Secondary plasmalogen deficiency disease states: respiratory disorders, Alzheimer disease.
► Plasmalogen replacement therapy.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1822, Issue 9, September 2012, Pages 1442–1452
نویسندگان
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