Cited by 1,082Lipid metabolism and biosynthesisMetabolism and Genetic DisordersFatty Acid Research and Health

Functions of plasmalogen lipids in health and disease

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Braverman, Nancy E., Moser, Ann B.·2012·Biochimica et Biophysica Acta (BBA) - Molecular Basis of 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.

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  • All RCDP patients share common symptoms irrespective of the affected gene.

    “Irrespective of the affected gene, all RCDP patients share common symptoms.”

    From Peroxisomes in brain development and function (2016) · cites this paper as [187]

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Authors
Braverman, N. E., & Moser, A. B.
Affiliations
  • Montreal Children's Hospital (CA)
  • Kennedy Krieger Institute (US)
Corresponding author
Braverman, Nancy E.
Venue
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Publisher
Elsevier BV
Published
2012-09
Volume
1822
Issue
9
Pages
1442-1452
ISSN
0925-4439 (ISSN-L)
OpenAlex
W1967190893
Cited by
1,082
References
124
Citations by year
2026: 84 · 2025: 88 · 2024: 82 · 2023: 90 · 2022: 100 · 2021: 110 · 2020: 114 · 2019: 90 · 2018: 76 · 2017: 61 · 2016: 61 · 2015: 52 · 2014: 42 · 2013: 25 · 2012: 6
FWCI
3.33
Type
journal article
Language
en
Open access
Locations
  • Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Montreal Childrens Hospital Foundation
  • Kennedy Krieger Institute Peroxisome Diseases Section
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
Keywords
plasmalogens, polyunsaturated fatty acids, plasmalogen biosynthesis, peroxisomes, rhizomelic chondrodysplasia punctata, tissue-specific function, lipid metabolism disorders
MeSH terms
Alzheimer Disease, Animals, Biological Transport, Cell Membrane, Glyceryl Ethers, Humans, Organ Specificity, Oxidation-Reduction, Plasmalogens, Respiratory Tract Diseases, Signal Transduction, Peroxisomal Disorders, Lipid Metabolism

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