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lüll Pancreas duodenum homeobox-1 regulates pancreas development during embryogenesis and islet cell function in adulthood Hui H; Perfetti REur J Endocrinol 2002[Feb]; 146 (2): 129-41Pancreas duodenum homeobox-1 (PDX-1) (also known as insulin promoter factor-1, islet/duodenum homeobox-1, somatostatin transactivating factor-1, insulin upstream factor-1 and glucose-sensitive factor) is a transcription factor encoded by a Hox-like homeodomain gene. In humans and other animal species, the embryonic development of the pancreas requires PDX-1, as demonstrated by the identification of an individual with pancreatic agenesis resulting from a mutation that impaired the transcription of a functionally active PDX-1 protein. In adult subjects, PDX-1 is essential for normal pancreatic islet function as suggested by its regulatory action on the expression of a number of pancreatic genes, including insulin, somatostatin, islet amyloid polypeptide, the glucose transporter type 2 and glucokinase. Furthermore, heterozygous mutations of PDX-1 have been linked to a type of autosomal dominant form of diabetes mellitus known as maturity onset diabetes of the young type 4. The dual action of PDX-1, as a differentiation factor during embryogenesis and as a regulator of islet cell physiology in mature islet cells, underscores the unique role of PDX-1 in health and disease of the human endocrine pancreas.|*Homeodomain Proteins[MESH]|Amino Acid Sequence[MESH]|Animals[MESH]|Cell Differentiation[MESH]|Cell Division[MESH]|Diabetes Mellitus, Type 2/genetics[MESH]|Diabetes Mellitus/genetics[MESH]|Gene Expression Regulation[MESH]|Humans[MESH]|Insulin/genetics[MESH]|Islets of Langerhans/cytology/*physiology[MESH]|Molecular Sequence Data[MESH]|Mutation[MESH]|Pancreas/*embryology[MESH]|Sequence Alignment[MESH]|Trans-Activators/chemistry/genetics/*physiology[MESH] |