Metabolism of Very Long-Chain Fatty Acids: Genes and Pathophysiology

Functional rafts in cell membranes

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The skin: an indispensable barrier

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Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters

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Global analysis of the yeast lipidome by quantitative shotgun mass spectrometry

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Fatty acid elongases in mammals: their regulation and roles in metabolism

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The role of omega-3 long-chain polyunsaturated fatty acids in health and disease of the retina

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The key roles of elongases and desaturases in mammalian fatty acid metabolism: Insights from transgenic mice

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Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

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Lipids of nervous tissue: Composition and metabolism

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Crucial role of a long-chain fatty acid elongase, Elovl6, in obesity-induced insulin resistance

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Essential fatty acids in visual and brain development

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Specialized pro-resolving lipid mediators in the inflammatory response: An update

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A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy

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Mutations in lipid transporter ABCA12 in harlequin ichthyosis and functional recovery by corrective gene transfer

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ELOVL1 production of C24 acyl-CoAs is linked to C24 sphingolipid synthesis

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Identification of a Mammalian Long Chain Fatty Acyl Elongase Regulated by Sterol Regulatory Element-binding Proteins

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Characterization of overall ceramide species in human stratum corneum

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Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas

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Loss of ceramide synthase 3 causes lethal skin barrier disruption

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X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects

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A Critical Role for Ceramide Synthase 2 in Liver Homeostasis

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A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy).

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Ceramide synthesis in the epidermis

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Ceramide biosynthesis in keratinocyte and its role in skin function

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Loss of functional ELOVL4 depletes very long-chain fatty acids (≥C28) and the unique ω-O-acylceramides in skin leading to neonatal death

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Deletion of ELOVL5 leads to fatty liver through activation of SREBP-1c in mice

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Laboratory findings in tear fluid analysis

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The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl–CoA esters

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Metabolic functions of peroxisomes in health and disease

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A Critical Role for Ceramide Synthase 2 in Liver Homeostasis

Pewzner‐Jung, Y., Brenner, O. J., Braun, S., Laviad, E. L., Ben‐Dor, S., Feldmesser, E., Horn‐Saban, S., Amann‐Zalcenstein, D., Raanan, C., Berkutzki, T., et al. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.077610 · Cited by 230 · see citing works

Meibomian Gland Diagnostic Expressibility: Correlation With Dry Eye Symptoms and Gland Location

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Mutations in the Fatty Acid 2-Hydroxylase Gene Are Associated with Leukodystrophy with Spastic Paraparesis and Dystonia

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Fatty acid 2-Hydroxylation in mammalian sphingolipid biology

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Identification of Two Mammalian Reductases Involved in the Two-carbon Fatty Acyl Elongation Cascade

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Dietary erucic acid therapy for X‐linked adrenoleukodystrophy

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Profiling and Characterizing Skin Ceramides Using Reversed-Phase Liquid Chromatography–Quadrupole Time-of-Flight Mass Spectrometry

t’Kindt, R., Jorge, L., Dumont, E., Couturon, P., David, F., Sandra, P., & Sandra, K. (2012) · Analytical Chemistry · doi:10.1021/ac202646v · Cited by 202 · see citing works

Role for ELOVL3 and Fatty Acid Chain Length in Development of Hair and Skin Function

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Recessive Mutations in ELOVL4 Cause Ichthyosis, Intellectual Disability, and Spastic Quadriplegia

Aldahmesh, M. A., Mohamed, J. Y., Alkuraya, H., Verma, I. C., Puri, R. D., Alaiya, A. A., Rizzo, W. B., & Alkuraya, F. S. (2011) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2011.10.011 · Cited by 186 · see citing works

Tear film lipids

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Peroxisomal ABC transporters: Structure, function and role in disease

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Human tear film and meibum. Very long chain wax esters and (O-acyl)-omega-hydroxy fatty acids of meibum

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The role of sphingolipid metabolism in cutaneous permeabilitybarrier formation

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The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy

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Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein

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Impaired Epidermal Permeability Barrier in Mice Lacking Elovl1 , the Gene Responsible for Very-Long-Chain Fatty Acid Production

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Impaired Epidermal Ceramide Synthesis Causes Autosomal Recessive Congenital Ichthyosis and Reveals the Importance of Ceramide Acyl Chain Length

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The complexity of sphingolipid biosynthesis in the endoplasmic reticulum

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Depletion of ceramides with very long chain fatty acids causes defective skin permeability barrier function, and neonatal lethality in ELOVL4 deficient mice

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Mutations in CERS3 Cause Autosomal Recessive Congenital Ichthyosis in Humans

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ELOVL2 controls the level of n-6 28:5 and 30:5 fatty acids in testis, a prerequisite for male fertility and sperm maturation in mice

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Very long chain (C24 to C36) polyenoic fatty acids of the n-3 and n-6 series in dipolyunsaturated phosphatidylcholines from bovine retina.

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SINE exonic insertion in the PTPLA gene leads to multiple splicing defects and segregates with the autosomal recessive centronuclear myopathy in dogs

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Functional roles of glycosphingolipids in signal transduction via lipid rafts

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Rescue and repair during photoreceptor cell renewal mediated by docosahexaenoic acid-derived neuroprotectin D1

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Ablation of Neuronal Ceramide Synthase 1 in Mice Decreases Ganglioside Levels and Expression of Myelin-associated Glycoprotein in Oligodendrocytes

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Characterization of four mammalian 3‐hydroxyacyl‐CoA dehydratases involved in very long‐chain fatty acid synthesis

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Unique lipids of primate spermatozoa: desmosterol and docosahexaenoic acid.

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Correlation of very long chain fatty acid accumulation and inflammatory disease progression in childhood X-ALD:

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Investigation of functions of essential fatty acids in the skin

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Male Germ Cells Require Polyenoic Sphingolipids with Complex Glycosylation for Completion of Meiosis

Rabionet, M., van der Spoel, A. C., Chuang, C., von Tümpling-Radosta, B., Litjens, M., Bouwmeester, D., Hellbusch, C. C., Körner, C., Wiegandt, H., Gorgas, K., et al. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m800870200 · Cited by 111 · see citing works

Photoreceptor outer segment phagocytosis attenuates oxidative stress-induced apoptosis with concomitant neuroprotectin D1 synthesis

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Involvement of very long fatty acid-containing lactosylceramide in lactosylceramide-mediated superoxide generation and migration in neutrophils

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Biochemical characterization of the very long-chain fatty acid elongase ELOVL7

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2-Hydroxy-ceramide synthesis by ceramide synthase family: enzymatic basis for the preference of FA chain length

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Exome sequencing reveals a novel mutation for autosomal recessive non-syndromic mental retardation in the TECR gene on chromosome 19p13

Çalışkan, M., Chong, J. X., Uricchio, L. H., Anderson, R. L., Chen, P., Sougnez, C. L., Garimella, K. V., Gabriel, S., DePristo, M. A., Shakir, K., et al. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddq569 · Cited by 105 · see citing works

A shift in sphingolipid composition from C24 to C16 increases susceptibility to apoptosis in HeLa cells

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Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2

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The roles of ABCA12 in epidermal lipid barrier formation and keratinocyte differentiation

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Essential Role of ELOVL4 Protein in Very Long Chain Fatty Acid Synthesis and Retinal Function

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Distinction between peroxisomal bifunctional enzyme and acyl‐CoA oxidase deficiencies

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Current and Future Pharmacological Treatment Strategies in X‐Linked Adrenoleukodystrophy

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Novel Class of Glycosphingolipids Involved in Male Fertility

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Very Long-chain Polyunsaturated Fatty Acids Are the Major Acyl Groups of Sphingomyelins and Ceramides in the Head of Mammalian Spermatozoa

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Ceramides and Sphingomyelins with High Proportions of Very Long-chain Polyunsaturated Fatty acids in Mammalian Germ Cells

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Essential fatty acids deficiency in humans

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Ablation of ceramide synthase 2 strongly affects biophysical properties of membranes

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Lorenzo's oil inhibits ELOVL1 and lowers the level of sphingomyelin with a saturated very long-chain fatty acid

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Genetics and molecular pathology of Stargardt-like macular degeneration

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The occurrence of polyenoic very long chain fatty acids with greater than 32 carbon atoms in molecular species of phosphatidylcholine in normal and peroxisome-deficient (Zellweger's syndrome) brain

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Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutants.

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Fatty acid composition of cholesteryl esters of human meibomian gland secretions

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Abnormal barrier function in the pathogenesis of ichthyosis: Therapeutic implications for lipid metabolic disorders

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Integrating genomics and transcriptomics to identify candidate genes for subcutaneous fat deposition in beef cattle

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Omega-9 monounsaturated fatty acids: a review of current scientific evidence of sources, metabolism, benefits, recommended intake, and edible safety

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Global assessment of oxidized free fatty acids in brain reveals an enzymatic predominance to oxidative signaling after trauma

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Comparative transcriptome analysis of the newly discovered insect vector of the pine wood nematode in China, revealing putative genes related to host plant adaptation

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Novel insights into brain lipid metabolism in Alzheimer's disease: Oligodendrocytes and white matter abnormalities

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Histological analyses by matrix-assisted laser desorption/ionization-imaging mass spectrometry reveal differential localization of sphingomyelin molecular species regulated by particular ceramide synthase in mouse brains

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Structure activity relationship (SAR) toxicological assessments: The role of expert judgment

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Seipin negatively regulates sphingolipid production at the ER–LD contact site

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Inherited disorders of complex lipid metabolism: A clinical review

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N-3 Polyunsaturated Fatty Acids as a Nutritional Support of the Reproductive and Immune System of Cattle—A Review

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Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1

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A Role for Fatty Acids in Peripheral Neuropathy Associated with Type 2 Diabetes and Prediabetes

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Cathepsin L Contributes to Reproductive Diapause by Regulating Lipid Storage and Survival of Coccinella septempunctata (Linnaeus)

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Characterization of Long-Chain Fatty Acid as N-(4-Aminomethylphenyl) Pyridinium Derivative by MALDI LIFT-TOF/TOF Mass Spectrometry

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Plasma phospholipid very-long-chain SFAs in midlife and 20-year cognitive change in the Atherosclerosis Risk in Communities (ARIC): a cohort study

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Alterations of Ultra Long-Chain Fatty Acids in Hereditary Skin Diseases—Review Article

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trans -2-Enoyl-CoA Reductase Tecr-Driven Lipid Metabolism in Endothelial Cells Protects against Transcytosis to Maintain Blood-Brain Barrier Homeostasis

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Two Modes of Regulation of the Fatty Acid Elongase ELOVL6 by the 3-Ketoacyl-CoA Reductase KAR in the Fatty Acid Elongation Cycle

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LINC00116-encoded microprotein mitoregulin regulates fatty acid metabolism at the mitochondrial outer membrane

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Hypomyelinating spastic dyskinesia and ichthyosis caused by a homozygous splice site mutation leading to exon skipping in ELOVL1

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The Role of Sirtuins in the Pathogenesis of Psoriasis

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Deciphering the modifiers for phenotypic variability of X-linked adrenoleukodystrophy

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The effect of combined supplementation with vitamin D and omega-3 fatty acids on blood glucose and blood lipid levels in patients with gestational diabetes

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Understanding the Roles of Very-Long-Chain Polyunsaturated Fatty Acids (VLC-PUFAs) in Eye Health

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Emerging roles for sphingolipids in cellular aging

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Serum lipids as an indicator for the alteration of liver function in patients with hepatitis B

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Development and Application of FASA, a Model for Quantifying Fatty Acid Metabolism Using Stable Isotope Labeling

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Growth and Fatty Acid Metabolism of Aphis gossypii Parasitized by the Parasitic Wasp Lysiphlebia japonica

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The role of very long chain fatty acids in yeast physiology and human diseases

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Lipids | Very Long Fatty Acids in Health and Disease

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Very long-chain fatty acids: elongation, physiology and related disorders

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Very long chain fatty acids in higher animals: a review

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The Suppression of Very Long Chain Fatty Acids Is Associated with Skin Carcinogenesis

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