Decreased fatty acit Â-oxidation in riboflavin-responsive, multiple acylocoenzyme a dehydrogenase-deficient patients is associated with an increase in uncoupling protein-3

Russell, Aaron, Schrauwen, Patrick, Somm, Emmanuel, Gastaldi, Giacomo, Hesselink, Matthijs, Schaart, Gert, Kornips, Esther, Lo, Sing Kai, Bufano, Daniela, Giacobino, Jean-Paul, Muzzin, Patrick, Ceccon, Mara, Angelini, Corrado and Vergani, Lodovica 2003, Decreased fatty acit Â-oxidation in riboflavin-responsive, multiple acylocoenzyme a dehydrogenase-deficient patients is associated with an increase in uncoupling protein-3, Journal of clinical endocrinology and metabolism, vol. 88, no. 12, pp. 5921-5926.

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Title Decreased fatty acit Â-oxidation in riboflavin-responsive, multiple acylocoenzyme a dehydrogenase-deficient patients is associated with an increase in uncoupling protein-3
Author(s) Russell, Aaron
Schrauwen, Patrick
Somm, Emmanuel
Gastaldi, Giacomo
Hesselink, Matthijs
Schaart, Gert
Kornips, Esther
Lo, Sing Kai
Bufano, Daniela
Giacobino, Jean-Paul
Muzzin, Patrick
Ceccon, Mara
Angelini, Corrado
Vergani, Lodovica
Journal name Journal of clinical endocrinology and metabolism
Volume number 88
Issue number 12
Start page 5921
End page 5926
Publisher The Endocrine Society
Place of publication Chevy Chase, MD
Publication date 2003
ISSN 0021-972X
1945-7197
Summary Riboflavin-responsive, multiple acylcoenzyme A dehydrogenase deficiency (RR-MAD), a lipid storage myopathy, is characterized by, among others, a decrease in fatty acid (FA) ß-oxidation capacity. Muscle uncoupling protein 3 (UCP3) is up-regulated under conditions that either increase the levels of circulating free FA and/or decrease FA ß-oxidation. Using a relatively large cohort of seven RR-MAD patients, we aimed to better characterize the metabolic disturbances of this disease and to explore the possibility that it might increase UCP3 expression. A battery of biochemical and molecular tests were performed, which demonstrated decreases in FA ß-oxidation and in the activities of respiratory chain complexes I and II. These metabolic alterations were associated with increases of 3.1- and 1.7-fold in UCP3 mRNA and protein expression, respectively. All parameters were restored to control values after riboflavin treatment. We postulate that the up-regulation of UCP3 in RR-MAD is due to the accumulation of muscle FA/acylCoA. RR-MAD is an optimal model to support the hypothesis that UCP3 is involved in the outward translocation of an excess of FA from the mitochondria and to show that, in humans, the effects of FA on UCP3 expression are direct and independent of fatty acid ß-oxidation.
Language eng
Field of Research 060199 Biochemistry and Cell Biology not elsewhere classified
Socio Economic Objective 970106 Expanding Knowledge in the Biological Sciences
HERDC Research category C1.1 Refereed article in a scholarly journal
Copyright notice ©2003 The Endocrine Society
Persistent URL http://hdl.handle.net/10536/DRO/DU:30004100

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