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Title: Thiamine pyrophosphate: an essential cofactor for the alpha-oxidation in mammals--implications for thiamine deficiencies?
Authors: Sniekers, Mieke
Foulon, Veerle
Mannaerts, Guy
Van Maldergem, L
Mandel, H
Gelb, B D
Casteels, Minne
Van Veldhoven, Paul P # ×
Issue Date: Jul-2006
Publisher: Birkhauser verlag ag
Series Title: Cellular and molecular life sciences : CMLS. vol:63 issue:13 pages:1553-63;
Abstract: The identification of 2-hydroxyphytanoyl-CoA lyase (2-HPCL), a thiamine pyrophosphate (TPP)-dependent peroxisomal enzyme involved in the alpha-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 alpha-oxidation has not been studied, nor have possible adverse effects of deficient alpha-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 alpha-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 alpha-oxidation. Whereas accumulation of phytanic acid and/or 2-hydroxyfatty acids or their alpha-oxidation intermediates in TRMA patients given a normal supply of thiamine is unlikely, this may not be true when malnourished.
URI: 
ISSN: 1420-682X
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Pharmacology Section (-)
Research Centre for Pharmaceutical Care and Pharmaco-economics (-)
Laboratory of Lipid Biochemistry and Protein Interactions
Department of Pharmaceutical & Pharmacological Sciences - miscellaneous
× corresponding author
# (joint) last author

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