PTF1 is a pancreas-specific protein that functions as a component of the trimeric pancreas transcription factor 1 (PTF1) complex. Localizing to the nucleus, PTF1 contains one basic helix-loop-helix (bHLH) domain and is believed to play an important role in mammalian pancreatic development, functioning as a transcription factor that regulates the specification of all three pancreatic cell types. PTF1 interacts with RBP-Jⅹ and, together, they cooperate in regulating the expression of PDX-1 (pancreas/duodenum homeobox protein 1), a key regulator of pancreatic islet development and insulin gene transcription in beta-cells. Loss of functional PTF1 can cause pancreatic progenitors to take on the normal fates of duodenal epithelia. Mutations in the gene encoding PTF1 lead to diabetes mellitus and cerebellar hypoplasia/agenesis, suggesting that PTF1 also plays and important role in cerebellar neurogenesis.