Heme regulates exocrine peptidase precursor genes in zebrafish.

摘要: We previously determined that yquem harbors a mutation in the gene encoding uroporphyrinogen decarboxylase (UROD), fifth enzyme heme biosynthesis, and established zebrafish (yqe(tp61)) as vertebrate model for human hepatoery-thropoietic porphyria (HEP). Here we report six exocrine peptidase precursor genes, carboxypeptidase A, trypsin precursor, like, chymotrypsinogen B1, 1-like, elastase 2 are downregulated yquem/urod (-/-), identified initially by microarray analysis of and, subsequently, confirmed situ hybridization. then downregulation these zymogens specifically pancreas sauternes (sau(tb223)) larvae, carrying delta-amino-levulinate synthase (ALAS2), first biosynthesis. also found ptf1a, transcription factor regulating zymogens, is both (-/-) sau/alas2 larvae. Further, hemin treatment rescues expression ptf1a sauternes/alas2 Thus, it appears deficiency downregulates which, turn, leads to zymogens. Our findings provide better understanding pathogenesis enhance our ability diagnose treat patients with or pancreatic diseases.