Risk factors for PDAC include a history of familial pancreatic cancer (FPC)8, hereditary syndromes predisposing to PDAC9, intraductal papillary mucinous neoplasms (IPMNs) and mucinous cystic neoplasms10, chronic pancreatitis11, (especially when related to inherited homologues of human cationic trypsinogen [PRSS1]), obesity12, new-onset diabetes (NOD)13 and smoking14. The gene discussed is PRSS1; the disease is dentatorubral-pallidoluysian atrophy.