mody 4 mody 4 mody 4 or pdx1-mody is a form of maturity onset diabetes of the young. mody 4specialtyendocrinology mody 4 arises from mutations of the pdx1 homeobox gene on chromosome 13. pdx-1 is a transcription factor vital to the development of the embryonic pancreas. even in adults it continues to play a role in the regulation and expression of genes for insulin, glut2, glucokinase, and somatostatin. mody 4 is so rare that only a single family has been well-studied. a child born with pancreatic agenesis (absence of the pancreas) was found to be homozygous for pdx-1 mutations. a number of older relatives who were heterozygous had mild hyperglycemia or diabetes. none were severely insulin-deficient and all were controlled with either diet or oral hypoglycemic agents.