Sjögren-Larsson syndrome: delta 5-and delta 6-fatty acid desaturases in skin fibroblasts. 1985

J Avigan, and B D Campbell, and D A Yost, and O Hernell, and G Holmgren, and S F Jagell

Cultured skin fibroblasts from two patients with Sjögren-Larsson syndrome (SLS) and from a normal control were analyzed for trienoic and tetraenoic fatty acids. In addition, we assayed desaturation of [1-14C]linoleic acid in cells from four patients and four controls. There was no significant effect of the disease on the composition of polyunsaturated fatty acids or on the rate of linoleic acid desaturation in fibroblasts. The results indicate the presence of delta 5- and delta 6-fatty acid desaturases in cells from SLS patients.

UI MeSH Term Description Entries
D007057 Ichthyosis Any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. Most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome. Xeroderma,Ichthyoses,Xerodermas
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D002851 Chromatography, High Pressure Liquid Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed. Chromatography, High Performance Liquid,Chromatography, High Speed Liquid,Chromatography, Liquid, High Pressure,HPLC,High Performance Liquid Chromatography,High-Performance Liquid Chromatography,UPLC,Ultra Performance Liquid Chromatography,Chromatography, High-Performance Liquid,High-Performance Liquid Chromatographies,Liquid Chromatography, High-Performance
D002855 Chromatography, Thin Layer Chromatography on thin layers of adsorbents rather than in columns. The adsorbent can be alumina, silica gel, silicates, charcoals, or cellulose. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed) Chromatography, Thin-Layer,Thin Layer Chromatography,Chromatographies, Thin Layer,Chromatographies, Thin-Layer,Thin Layer Chromatographies,Thin-Layer Chromatographies,Thin-Layer Chromatography
D005260 Female Females
D005347 Fibroblasts Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. Fibroblast
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000090943 Delta-5 Fatty Acid Desaturase A fatty acid desaturase that is a rate-limiting enzyme for the conversion from dihomo-gamma-linolenic acid and EICOSATETRAENOATE (ETA) in the synthesis of ARACHIDONIC ACID and EICOSAPENTAENOATE (EPA). It is also involved in the biosynthesis of unsaturated fatty acids from the polyunsaturated fatty acids (PUFA), LINOLEIC ACID (LA), and ALPHA LINOLENIC ACID (ALA) precursors and plays an important role in the metabolism of inflammatory lipids including PROSTAGLANDIN E2. Delta 5 Desaturase,Delta-5-FADase,Fatty Acid Delta-5-Desaturase,5 Desaturase, Delta,Acid Delta-5-Desaturase, Fatty,Delta 5 FADase,Delta-5-Desaturase, Fatty Acid,Desaturase, Delta 5,Fatty Acid Delta 5 Desaturase
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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