title: Functions of plasmalogen lipids in health and disease.
abstract: 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.
authors.affiliation: Department of Human Genetics and Pediatrics, McGill University-Montreal Childrens Hospital Research Institute, Montreal, Canada. nancy.braverman@mcgill.ca
authors.forename: Nancy E
authors.initials: NE
authors.lastname: Braverman
authors.forename: Ann B
authors.initials: AB
authors.lastname: Moser
mesh_terms: D000544:Alzheimer Disease / Q000378:metabolism; D000818:Animals; D001692:Biological Transport; D002462:Cell Membrane / Q000378:metabolism; D005995:Glyceryl Ethers / Q000627:therapeutic use; D006801:Humans; D050356:Lipid Metabolism; D009928:Organ Specificity; D010084:Oxidation-Reduction; D018901:Peroxisomal Disorders / Q000188:drug therapy / Q000378:metabolism*; D010955:Plasmalogens / Q000096:biosynthesis / Q000378:metabolism / Q000502:physiology*; D012140:Respiratory Tract Diseases / Q000378:metabolism; D015398:Signal Transduction
chemical_list: D005995:Glyceryl Ethers; D010955:Plasmalogens; C033433:batyl alcohol