Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids
| Autor: | Maria S.K. Stoll, Paul E. Minkler, Henk van Lenthe, Sander M. Houten, Charles L. Hoppel, Wilhelm Stoffel, Jörn Oliver Sass, Ronald J.A. Wanders, K Otfried Schwab, Uwe Janssen, Wim Kulik, Heleen te Brinke, Michel van Weeghel |
|---|---|
| Přispěvatelé: | University of Zurich, Houten, Sander M, Other departments, Amsterdam Gastroenterology Endocrinology Metabolism, Laboratory Genetic Metabolic Diseases, Paediatric Metabolic Diseases |
| Rok vydání: | 2012 |
| Předmět: |
Blood Glucose
medicine.medical_specialty 1303 Biochemistry Immunoblotting 610 Medicine & health Isomerase Mitochondrion Biology Dodecenoyl-CoA Isomerase Real-Time Polymerase Chain Reaction Biochemistry Mass Spectrometry Mice 1311 Genetics Internal medicine Carnitine 1312 Molecular Biology Genetics medicine Animals Molecular Biology Incubation Beta oxidation Unsaturated fatty acid Cells Cultured Chromatography High Pressure Liquid chemistry.chemical_classification Mice Knockout Gene knockdown Carbon-Carbon Double Bond Isomerases Mitochondria Enzyme Endocrinology chemistry 10036 Medical Clinic 1305 Biotechnology Fatty Acids Unsaturated Oxidation-Reduction Biotechnology |
| Zdroj: | FASEB journal, 26(10), 4316-4326. FASEB |
| ISSN: | 1530-6860 0892-6638 |
| Popis: | Mitochondrial enoyl-CoA isomerase (ECI1) is an auxiliary enzyme involved in unsaturated fatty acid oxidation. In contrast to most of the other enzymes involved in fatty acid oxidation, a deficiency of ECI1 has yet to be identified in humans. We used wild-type (WT) and Eci1-deficient knockout (KO) mice to explore a potential presentation of human ECI1 deficiency. Upon food withdrawal, Eci1-deficient mice displayed normal blood beta-hydroxybutyrate levels (WT 1.09 mM vs. KO 1.10 mM), a trend to lower blood glucose levels (WT 4.58 mM vs. KO 3.87 mM, P=0.09) and elevated blood levels of unsaturated acylcarnitines, in particular C12:1 acylcarnitine (WT 0.03 mu M vs. KO 0.09 mu M, P |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text