Pancreas duodenum homeobox-1 regulates pancreas development during embryogenesis and islet cell function in adulthood

Eur J Endocrinol. 2002 Feb;146(2):129-41. doi: 10.1530/eje.0.1460129.


Pancreas 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.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Cell Differentiation
  • Cell Division
  • Diabetes Mellitus / genetics
  • Diabetes Mellitus, Type 2 / genetics
  • Gene Expression Regulation
  • Homeodomain Proteins*
  • Humans
  • Insulin / genetics
  • Islets of Langerhans / cytology
  • Islets of Langerhans / physiology*
  • Molecular Sequence Data
  • Mutation
  • Pancreas / embryology*
  • Sequence Alignment
  • Trans-Activators / chemistry
  • Trans-Activators / genetics
  • Trans-Activators / physiology*


  • Homeodomain Proteins
  • Insulin
  • Trans-Activators
  • pancreatic and duodenal homeobox 1 protein