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Diabetes in friedreich ataxia.
MedLine Citation:
PMID:  23859345     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Diabetes is a common metabolic disorder in patients with Friedreich ataxia. In this Supplement article, we review the clinical data on diabetes in Friedreich ataxia, and the experimental data from rodent and in vitro models of the disease. Increased body adiposity and insulin resistance are frequently present in Friedreich ataxia, but pancreatic β cell dysfunction and death are a conditio sine qua non for the loss of glucose tolerance and development of diabetes. The loss of frataxin function in mitochondria accounts for these pathogenic processes in Friedreich ataxia. Mitochondria are essential for the sensing of nutrients by the β cell and for the generation of signals that trigger and amplify insulin secretion, known as stimulus-secretion coupling. Moreover, in the intrinsic pathway of apoptosis, pro-apoptotic signals converge on mitochondria, resulting in mitochondrial Bax translocation, membrane permeabilization, cytochrome c release and caspase cleavage. How and at which level frataxin deficiency impacts on these processes in β cells is only partially understood. A better understanding of the molecular mechanisms mediating β cell demise in Friedreich ataxia will pave the way for new therapeutic approaches.
Authors:
Miriam Cnop; Hindrik Mulder; Mariana Igoillo-Esteve
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of neurochemistry     Volume:  126 Suppl 1     ISSN:  1471-4159     ISO Abbreviation:  J. Neurochem.     Publication Date:  2013 Aug 
Date Detail:
Created Date:  2013-07-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985190R     Medline TA:  J Neurochem     Country:  England    
Other Details:
Languages:  eng     Pagination:  94-102     Citation Subset:  IM    
Copyright Information:
© 2013 International Society for Neurochemistry.
Affiliation:
Laboratory of Experimental Medicine, Université Libre de Bruxelles, Brussels, Belgium; Division of Endocrinology, Erasmus Hospital, Brussels, Belgium.
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