mutations in ucp2 in congenital hyperinsulinism reveal a role for regulation of insulin secretion突变ucp2在先天性高胰岛素症显示调节胰岛素分泌的作用.pdfVIP
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mutations in ucp2 in congenital hyperinsulinism reveal a role for regulation of insulin secretion突变ucp2在先天性高胰岛素症显示调节胰岛素分泌的作用
Mutations in UCP2 in Congenital Hyperinsulinism Reveal a Role for Regulation of Insulin Secretion ´ 1,3 2 ´ 1 3 ´ M. Mar Gonzalez-Barroso , Irina Giurgea , Frederic Bouillaud , Andrea Anedda , Christine Bellanne- 4 2 2 2 1 Chantelot , Laurence Hubert , Yves de Keyzer , Pascale de Lonlay , Daniel Ricquier * ´ ´ 1 Universite Paris Descartes Site Necker-enfants Malades, CNRS UPR 9078 BIOTRAM, Paris, France, 2 Universite Paris Descartes Site Necker-enfants Malades, INSERM U781 ˆ ˆ ´ and Departments of Pediatrics, Hopital Necker-Enfants Malades Assistance Publique Hopitaux de Paris, Paris, France, 3 Centro de Investigaciones Biologicas, CSIC, Madrid, ´ ´ ´ ´ ´ ` ˆ Spain, 4 Departement de Genetique, Groupe Hospitalier Pitie-Salpetriere Assistance Publique Hopitaux de Paris, Paris, France Abstract Although the most common mechanism underlying congenital hyperinsulinism is dysfunction of the pancreatic ATP- sensitive potassium channel, the pathogenesis and genetic origins of this disease remains largely unexplained in more than half of all patients. UCP2 knockout mice exhibit an hyperinsulinemic hypoglycemia, suggesting an involment of UCP2 in insulin secretion. However, a possible pathogenic role for UCP2 protein in the development of huma
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