Thiamine pyrophosphate: An essential cofactor for the α-oxidation in mammals – implications for thiamine deficiencies?
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The identification of 2-hydroxyphytanoyl-CoA lyase (2-HPCL), a thiamine pyrophosphate (TPP)-dependent peroxisomal enzyme involved in the α-oxidation of phytanic acid and of 2-hydroxy straight chain fatty acids, pointed towards a role of TPP in these processes. Until then, TPP had not been implicated in mammalian peroxisomal metabolism. The effect of thiamine deficiency on 2-HPCL and α-oxidation has not been studied, nor have possible adverse effects of deficient α-oxidation been considered in the pathogenesis of diseases associated with thiamine shortage, such as thiamine-responsive megaloblastic anemia (TRMA). Experiments with cultured cells and animal models showed that α-oxidation is controlled by the thiamine status of the cell/tissue/organism, and suggested that some pathological consequences of thiamine starvation could be related to impaired α-oxidation. Whereas accumulation of phytanic acid and/or 2-hydroxyfatty acids or their α-oxidation intermediates in TRMA patients given a normal supply of thiamine is unlikely, this may not be true when malnourished.
- Thiamine pyrophosphate: An essential cofactor for the α-oxidation in mammals – implications for thiamine deficiencies?
Cellular and Molecular Life Sciences CMLS
Volume 63, Issue 13 , pp 1553-1563
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- 2-hydroxyfatty acids
- phytanic acid
- thiamine-responsive megaloblastic anemia
- vitamin B
- Wernicke-Korsakoff syndrome
- Industry Sectors
- Author Affiliations
- 1. Departement Moleculaire Celbiologie, Afdeling Farmacologie, Faculteit Geneeskunde, Katholieke Universiteit Leuven, O & N1, Herestraat 49, Box 601, 3000, Leuven, Belgium
- 2. Centre de Génétique Humaine – Maladies métabolique, Université de Liège, CHU Sart-Tilman, 4000, Liège, Belgium
- 3. Metabolic Unit, Department of Pediatrics, Rambam Medical Center, Haifa, Israel
- 4. Departments of Pediatrics and Human Genetics, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY, 10029, USA