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Horm Metab Res 2006; 38(10): 683-687
DOI: 10.1055/s-2006-954583
Original Clinical

© Georg Thieme Verlag KG Stuttgart · New York

Regular Insulin Secretory Oscillations Despite Impaired ATP Synthesis in Friedreich Ataxia Patients

C. Meyer 1 , H. Carlqvist 2 , M. Vorgerd 3 , L. Schöls 1 , 4 , C.-G. Östenson 5 , M. Ristow 6 , 7
  • 1Department of Neurology, St. Josef Hospital, Ruhr-University Bochum, Germany
  • 2Department of Mathematics, Royal Institute of Technology (KTH), Stockholm, Sweden
  • 3Department of Neurology, Kliniken Bergmannsheil, Ruhr-University Bochum, Germany
  • 4Current address: Research Division for Clinical Neurogenetics, Centre of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Germany
  • 5Department of Molecular Medicine, Endocrine and Diabetes Unit, Karolinska Institute, Stockholm, Sweden
  • 6German Institute for Human Nutrition and Charité University Medicine, CBF, Berlin, Germany
  • 7Current address: Department of Human Nutrition, Inst. of Nutrition, University of Jena, Germany
Further Information

Publication History

Received 9 February 2006

Accepted after revision 30 May 2006

Publication Date:
30 October 2006 (online)

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Abstract

Friedreich Ataxia is an inherited disorder caused by decreased expression of a mitochondrial protein called frataxin. Deficiency of this protein causes reduced biogenesis of iron-sulfur clusters, and subsequently impaired synthesis and replenishment of ATP in vivo. Basal secretion of insulin occurs in an oscillating manner presumably triggered by ATP-dependent feedback inhibition of glycolytic flux. Hence, individuals with reduced ATP synthesis rates should possibly exhibit impaired insulin secretory oscillations if these were solely dependent on ATP. In the present study Friedreich Ataxia patients with a presumptive impairment of ATP synthesis in pancreatic beta-cells were evaluated for regularity of basal secretory oscillations of insulin. Healthy siblings were employed as controls. In conflict with the initial hypothesis, no differences in regards to oscillation patterns were observed between patients and controls. Supported by ex vivo evidence, these findings tentatively suggest that pulsatile insulin secretion might not be exclusively dependent on ATP feedback inhibition in humans.

Key words

Frataxin - pulsatile insulin secretion - oxidative phosphorylation - ATP - type 2 diabetes

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Correspondence

Michael Ristow

Department of Human Nutrition·Institute of Nutrition·University of Jena·29 Dornburger St.·07743 Jena·Germany

Phone: +49/3641/94 96 30

Fax: +49/3641/94 96 32

Email: michael.ristow@mristow.org

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