Question: How is the PRSS1 gene related to hereditary pancreatitis?

Gaia Andreoletti answered on 7 Mar 2016:

Mutations in the PRSS1 gene cause most cases of hereditary pancreatitis. The PRSS1 gene provides instructions for making an enzyme, called cationic trypsinogen, which helps with the digestion of food.

It is estimated that 65 to 80 percent of people with hereditary pancreatitis have mutations in the PRSS1 gene. The remaining cases are caused by mutations in other three known genes, and some of the disease causal genes have not been identified yet.

When hereditary pancreatitis is caused by mutations in the PRSS1 gene, it is inherited in an autosomal dominant pattern, which means that an affected person inherits the PRSS1 gene mutation from one affected parent.