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Essential role of Pax3 in islet progenitor cell development.
The pancreas consists of two classes of endocrine cells, the α and β cells. Pax6 is essential for the differentiation of endocrine cells from pancreatic progenitor cells and the early function of Pdx1 in β-cell specification. Pax3, a relative of Pax6, is expressed in early pancreatic cells, and plays a role in development of endocrine cells, and a few β cells in the dorsal pancreas. However, its contribution to the endocrine and exocrine lineages in the adult pancreas is unclear. In this study, we found that Pax3 is expressed in adult pancreatic progenitor cells, and partially overlaps with insulin (Ins), glucagon (Gcg), somatostatin (Sst), pancreatic polypeptide (Ppy), pancreatic and duodenal homeobox 1 (Pdx1), and nestin, markers of progenitor/stem cells, and Pdx1(+)/insulin(+)/glucagon(+)/somatostatin(+) cells in the adult pancreas. Interestingly, Pax3-deficient mice showed severe diabetes, including a reduction in pancreas mass and histological disorganization of islets. However, the number and size of Pdx1(+)/insulin(+) cells in the duodenum were normal in Pax3-deficient mice. These results indicate that Pax3 is expressed in pancreatic stem
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