TY - JOUR
T1 - Functions of plasmalogen lipids in health and disease
AU - Braverman, Nancy E.
AU - Moser, Ann B.
N1 - Funding Information:
We would like to acknowledge the late Dr. Hugo Moser, whose foresight enabled the establishment of PBD patient cell lines from which we have learned so much. We also wish to thank the parent organizations ( www.Rhizokids.org and www.theGFPD.org ) for their contributions to further knowledge of these disorders. NEB is supported by the Montreal Childrens Hospital Foundation and ABM is supported by the Kennedy Krieger Institute Peroxisome Diseases Section .
PY - 2012/9
Y1 - 2012/9
N2 - 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.
AB - 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.
KW - Alzheimer disease
KW - Lipid signaling
KW - Plasmalogen
KW - Plasmalogen replacement therapy
KW - Respiratory disease
KW - Rhizomelic Chondrodysplasia Punctata
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U2 - 10.1016/j.bbadis.2012.05.008
DO - 10.1016/j.bbadis.2012.05.008
M3 - Review article
C2 - 22627108
AN - SCOPUS:84864046701
SN - 0925-4439
VL - 1822
SP - 1442
EP - 1452
JO - Biochimica et Biophysica Acta - Molecular Basis of Disease
JF - Biochimica et Biophysica Acta - Molecular Basis of Disease
IS - 9
ER -