ReviewJournal of inherited metabolic disease2015
Plasmalogens and fatty alcohols in rhizomelic chondrodysplasia punctata and Sjögren-Larsson syndrome.
Review in Journal of inherited metabolic disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 39 citations in OpenAlex.
- Plasmalogen as a Bioactive Lipid Drug: From Preclinical Research Challenges to Opportunities in Nanomedicine.FASEB bioAdvances · 2025Article
- The neurological pathology of peroxisomal ACBD5 deficiency - lessons from patients and mouse models.Frontiers in molecular neuroscience · 2025Review
- An adaptive stress response that confers cellular resilience to decreased ubiquitination.Nature communications · 2023Article
- Vitamin B3 Biotech · 2023Article
- TRIM21 attenuates renal carcinoma lipogenesis and malignancy by regulating SREBF1 protein stability.Journal of experimental & clinical cancer research : CR · 2023Article
- The emerging roles of TRIM21 in coordinating cancer metabolism, immunity and cancer treatment.Frontiers in immunology · 2022Review
- ATP8B2-Mediated Asymmetric Distribution of Plasmalogens Regulates Plasmalogen Homeostasis and Plays a Role in Intracellular Signaling.Frontiers in molecular biosciences · 2022Article
- Article
- Peroxisomal Disorders and Their Mouse Models Point to Essential Roles of Peroxisomes for Retinal Integrity.International journal of molecular sciences · 2021Review
- Cerebellar and hepatic alterations in ACBD5-deficient mice are associated with unexpected, distinct alterations in cellular lipid homeostasis.Communications biology · 2020Article
- Enhancing detection and characterization of lipids using charge manipulation in electrospray ionization-tandem mass spectrometry.Chemistry and physics of lipids · 2020Review
- Structural Elucidation of Ether Glycerophospholipids Using Gas-Phase Ion/Ion Charge Inversion Chemistry.Journal of the American Society for Mass Spectrometry · 2020Article
- Stabilization of FASN by ACAT1-mediated GNPAT acetylation promotes lipid metabolism and hepatocarcinogenesis.Oncogene · 2020Article
- Peroxisomal Dysfunction in Neurological Diseases and Brain Aging.Frontiers in cellular neuroscience · 2020Article
- Plasmalogen loss caused by remodeling deficiency in mitochondria.Life science alliance · 2019Article
- Understanding the diversity of membrane lipid composition.Nature reviews. Molecular cell biology · 2018Review
- Substantial Decrease in Plasmalogen in the Heart Associated with Tafazzin Deficiency.Biochemistry · 2018Article
- 2-Chlorofatty acids induce Weibel-Palade body mobilization.Journal of lipid research · 2018Article
- Protective role of endogenous plasmalogens against hepatic steatosis and steatohepatitis in mice.Hepatology (Baltimore, Md.) · 2017Article
- Metabolic Interplay between Peroxisomes and Other Subcellular Organelles Including Mitochondria and the Endoplasmic Reticulum.Frontiers in cell and developmental biology · 2015Review
Corrections and comments
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Authors and funding
3 authors at 1 institution.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Plasmalogens are a special class of ether-phospholipids, best recognized by their vinyl-ether bond at the sn-1 position of the glycerobackbone and by the observation that their deficiency causes rhizomelic chondrodysplasia punctata (RCDP). The complex plasmalogen biosynthetic pathway involves multiple enzymatic steps carried-out in peroxisomes and in the endoplasmic reticulum. The rate limiting step in the biosynthesis of plasmalogens resides in the formation of the fatty alcohol responsible for the formation of an intermediate with an alkyl-linked moiety. The regulation in the biosynthesis of plasmalogens also takes place at this step using a feedback mechanism to stimulate or inhibit the biosynthesis. As such, fatty alcohols play a relevant role in the formation of ether-phospholipids. These advances in our understanding of complex lipid biosynthesis brought two seemingly distinct disorders into the spotlight. Sjögren-Larsson syndrome (SLS) is caused by defects in the microsomal fatty aldehyde dehydrogenase (FALDH) leading to the accumulation of fatty alcohols and fatty aldehydes. In RCDP cells, the defect in plasmalogens is thought to generate a feedback signal to increase their biosynthesis, through the activity of fatty acid reductases to produce fatty alcohols. However, the enzymatic defects in either glyceronephosphate O-acyltransferase (GNPAT) or alkylglycerone phosphate synthase (AGPS) disrupt the biosynthesis and result in the accumulation of the fatty alcohols. A detailed characterization on the processes and enzymes that govern these intricate biosynthetic pathways, as well as, the metabolic characterization of defects along the pathway should increase our understanding of the causes and mechanisms behind these disorders.
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