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

Journal of biochemistry (Tokyo), 2012-11, Vol.152 (5), p.387-395

Kihara, Akio

2012

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Comparative profiling and comprehensive quantification of stratum corneum ceramides in humans and mice by LC/MS/MS[S]

Journal of lipid research, 2020-06, Vol.61 (6), p.884-895

2020

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PNPLA1 is a transacylase essential for the generation of the skin barrier lipid ω-O-acylceramide

Nature communications, 2017-03, Vol.8 (1), p.14610-14610, Article 14610

2017

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Lipid polarity gradient formed by ω-hydroxy lipids in tear film prevents dry eye disease

eLife, 2020-04, Vol.9

2020

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Enzyme Activities of the Ceramide Synthases CERS2–6 Are Regulated by Phosphorylation in the C-terminal Region

The Journal of biological chemistry, 2016-04, Vol.291 (14), p.7477-7487

2016

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Alteration of epidermal lipid composition as a result of deficiency in the magnesium transporter Nipal4

Journal of lipid research, 2024-06, Vol.65 (6), p.100550, Article 100550

2024

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Impaired production of the skin barrier lipid acylceramide by CYP4F22 ichthyosis mutations

Journal of dermatological science, 2021-01, Vol.101 (1), p.69-71

2021

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The Rim101 pathway contributes to ER stress adaptation through sensing the state of plasma membrane

Biochemical journal, 2017-01, Vol.474 (1), p.51-63

2017

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Impaired production of skin barrier lipid acylceramides and abnormal localization of PNPLA1 due to ichthyosis-causing mutations in PNPLA1

Journal of dermatological science, 2022-08, Vol.107 (2), p.89-94

2022

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

Proceedings of the National Academy of Sciences - PNAS, 2010-10, Vol.107 (43), p.18439-18444

2010

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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

PloS one, 2014-07, Vol.9 (7), p.e101823-e101823

2014

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Mammalian Lass6 and its related family members regulate synthesis of specific ceramides

Biochemical journal, 2005-08, Vol.390 (Pt 1), p.263-271

2005

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Identification of acyl-CoA synthetases involved in the mammalian sphingosine 1-phosphate metabolic pathway

Biochemical and biophysical research communications, 2013-12, Vol.442 (3-4), p.195-201

2013

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Membrane Protein Rim21 Plays a Central Role in Sensing Ambient pH in Saccharomyces cerevisiae

The Journal of biological chemistry, 2012-11, Vol.287 (46), p.38473-38481

2012

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

Journal of lipid research, 2014-03, Vol.55 (3), p.524-530

2014

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Dual Functions of the Trans-2-Enoyl-CoA Reductase TER in the Sphingosine 1-Phosphate Metabolic Pathway and in Fatty Acid Elongation

The Journal of biological chemistry, 2014-09, Vol.289 (36), p.24736-24748

2014

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The 3-hydroxyacyl-CoA dehydratases HACD1 and HACD2 exhibit functional redundancy and are active in a wide range of fatty acid elongation pathways

The Journal of biological chemistry, 2017-09, Vol.292 (37), p.15538-15551

2017

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Decreased Skin Barrier Lipid Acylceramide and Differentiation-Dependent Gene Expression in Ichthyosis Gene Nipal4-Knockout Mice

Journal of investigative dermatology, 2018-04, Vol.138 (4), p.741-749

2018

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Identification of the phytosphingosine metabolic pathway leading to odd-numbered fatty acids

Nature communications, 2014-10, Vol.5 (1), p.5338-5338, Article 5338

2014

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Mechanistic Details of Early Steps in Coenzyme Q Biosynthesis Pathway in Yeast

Cell chemical biology, 2016-10, Vol.23 (10), p.1241-1250

2016

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Metabolism and biological functions of two phosphorylated sphingolipids, sphingosine 1-phosphate and ceramide 1-phosphate

Progress in lipid research, 2007-03, Vol.46 (2), p.126-144

2007

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Catalytic residues, substrate specificity, and role in carbon starvation of the 2-hydroxy FA dioxygenase Mpo1 in yeast

Journal of lipid research, 2020-07, Vol.61 (7), p.1104-1114

2020

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Ceramide Analysis in Combination With Genetic Testing May Provide a Precise Diagnosis for Self-Healing Collodion Babies

Journal of lipid research, 2022-12, Vol.63 (12), p.100308-100308, Article 100308

2022

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AM251 Suppresses Epithelial-Mesenchymal Transition of Renal Tubular Epithelial Cells

PloS one, 2016-12, Vol.11 (12), p.e0167848

2016

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Loop 5 region is important for the activity of the long-chain base transporter Rsb1

Journal of biochemistry (Tokyo), 2017-02, Vol.161 (2), p.207-213

2017

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

Comparative profiling and comprehensive quantification of stratum corneum ceramides in humans and mice by LC/MS/MS[S]

PNPLA1 is a transacylase essential for the generation of the skin barrier lipid ω-O-acylceramide

Lipid polarity gradient formed by ω-hydroxy lipids in tear film prevents dry eye disease

Enzyme Activities of the Ceramide Synthases CERS2–6 Are Regulated by Phosphorylation in the C-terminal Region

Alteration of epidermal lipid composition as a result of deficiency in the magnesium transporter Nipal4

Impaired production of the skin barrier lipid acylceramide by CYP4F22 ichthyosis mutations

The Rim101 pathway contributes to ER stress adaptation through sensing the state of plasma membrane

Impaired production of skin barrier lipid acylceramides and abnormal localization of PNPLA1 due to ichthyosis-causing mutations in PNPLA1

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

Two modes of regulation of the fatty acid elongase ELOVL6 by the 3-ketoacyl-CoA reductase KAR in the fatty acid elongation cycle

Mammalian Lass6 and its related family members regulate synthesis of specific ceramides

Identification of acyl-CoA synthetases involved in the mammalian sphingosine 1-phosphate metabolic pathway

Membrane Protein Rim21 Plays a Central Role in Sensing Ambient pH in Saccharomyces cerevisiae

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

Dual Functions of the Trans-2-Enoyl-CoA Reductase TER in the Sphingosine 1-Phosphate Metabolic Pathway and in Fatty Acid Elongation

The 3-hydroxyacyl-CoA dehydratases HACD1 and HACD2 exhibit functional redundancy and are active in a wide range of fatty acid elongation pathways

Decreased Skin Barrier Lipid Acylceramide and Differentiation-Dependent Gene Expression in Ichthyosis Gene Nipal4-Knockout Mice

Identification of the phytosphingosine metabolic pathway leading to odd-numbered fatty acids

Mechanistic Details of Early Steps in Coenzyme Q Biosynthesis Pathway in Yeast

Metabolism and biological functions of two phosphorylated sphingolipids, sphingosine 1-phosphate and ceramide 1-phosphate

Catalytic residues, substrate specificity, and role in carbon starvation of the 2-hydroxy FA dioxygenase Mpo1 in yeast

Ceramide Analysis in Combination With Genetic Testing May Provide a Precise Diagnosis for Self-Healing Collodion Babies

AM251 Suppresses Epithelial-Mesenchymal Transition of Renal Tubular Epithelial Cells

Loop 5 region is important for the activity of the long-chain base transporter Rsb1

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