Article
Mitochondrial dysfunction contributes to impaired insulin secretion in INS-1 cells with dominant-negative mutations of HNF-1alpha and in HNF-1alpha-deficient islets.
The Journal of biological chemistry - 19 Jun 2009
Pongratz Rebecca L, Kibbey Richard G, Kirkpatrick Clare L, Zhao Xiaojian, Pontoglio Marco, Yaniv Moshe, Wollheim Claes B, Shulman Gerald I, Cline Gary W
Abstract excerpt
Maturity Onset Diabetes of the Young-type 3 (MODY-3) has been linked to mutations in the transcription factor hepatic nuclear factor (HNF)-1alpha, resulting in deficiency in glucose-stimulated insulin secretion. In INS-1 cells overexpressing doxycycline-inducible HNF-1alpha dominant-negative (DN-) gene mutations, and islets from Hnf-1alpha knock-out mice, insulin secretion was impaired in response to glucose (15...
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