Functions of plasmalogen lipids in health and disease
Open accessAbstract
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
- bronze
- doi.org
- Licence
- 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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