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Frataxin deficiency in pancreatic islets causes diabetes due to loss of β cell mass
Michael Ristow, … , Michel Koenig, Andreas F.H. Pfeiffer
Michael Ristow, … , Michel Koenig, Andreas F.H. Pfeiffer
Published August 15, 2003
Citation Information: J Clin Invest. 2003;112(4):527-534. https://doi.org/10.1172/JCI18107.
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Article Metabolism

Frataxin deficiency in pancreatic islets causes diabetes due to loss of β cell mass

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Abstract

Diabetes is caused by an absolute (type 1) or relative (type 2) deficiency of insulin-producing β cells. We have disrupted expression of the mitochondrial protein frataxin selectively in pancreatic β cells. Mice were born healthy but subsequently developed impaired glucose tolerance progressing to overt diabetes mellitus. These observations were explained by impairment of insulin secretion due to a loss of β cell mass in knockout animals. This phenotype was preceded by elevated levels of reactive oxygen species in knockout islets, an increased frequency of apoptosis, and a decreased number of proliferating β cells. Hence, disruption of the frataxin gene in pancreatic β cells causes diabetes following cellular growth arrest and apoptosis, paralleled by an increase in reactive oxygen species in islets. These observations might provide insight into the deterioration of β cell function observed in different subtypes of diabetes in humans.

Authors

Michael Ristow, Hindrik Mulder, Doreen Pomplun, Tim J. Schulz, Katrin Müller-Schmehl, Anja Krause, Malin Fex, Hélène Puccio, Jörg Müller, Frank Isken, Joachim Spranger, Dirk Müller-Wieland, Mark A. Magnuson, Matthias Möhlig, Michel Koenig, Andreas F.H. Pfeiffer

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Figure 3

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Diabetes develops because of decreased insulin secretion caused by a pro...
Diabetes develops because of decreased insulin secretion caused by a progressive reduction in β cell mass. (a) β-hydroxybutyrate levels in knockout animals at different ages. (b) Results of an insulin-tolerance test at an age of 18 weeks. (c) Blood glucose levels 2 hours after intraperitoneal (i.p.) injection of glucose. (d) Insulin levels after intraperitoneal injection of glucose in 18-week-old mice. (e) Static incubations of isolated islets from 6-week-old animals in 35 mM potassium chloride and different concentrations of glucose (as indicated). (f) Number of islets isolated by collagenase digestion from pancreata of mice at different ages. (g) Number of insulin-positive areas on all pancreatic sections per pancreas from mice at different ages. (h) Ratios of total insulin-positive area in pancreata from mice at different ages per whole pancreatic area. (i) Average size per insulin-positive area from mice at different ages. Black bars, knockout animals; gray bars, control animals. (j) Subsets of microphotographs (original magnification, ×40) from mice evaluated in g, using an anti-insulin antibody. Error bars depict SEM. *0.05 > P > 0.005; **P < 0.005.

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