Table 1.

Common mutations in patients with hereditary pancreatitis.

Gene	Mechanism of action	Inheritance
PRSS1	Mutations in the serine protease 1 gene (PRSS) which encodes cationic trypsinogen can lead to increased trypsin activity, increased trypsin stability, and increased autoactivation	Autosomal dominant
SPINK1	SPINK1 encodes a pancreatic secretory trypsin inhibitor, mutations interfere with the protective function and predispose a person to pancreatitis possibly via increased intrapancreatic trypsin activity	Complex inheritance pattern
CFTR	Mutation causes a defect in the CFTR protein which causes abnormal sodium and chloride transport, leading to defective pancreatic secretion	Autosomal recessive

SPINK1, serine protease inhibitor Kazal type 1; CFTR, cystic fibrosis transmembrane conductance regulator.