Dubin-Johnson syndrome (DJS) is an inherited disorder of bilirubin characterized by a buildup of bilirubin in the bloodstream (hyperbilirubinemia). When bilirubin builds up in the skin and the whites of the eyes, it can cause a yellowish color to the skin (jaundice). Individuals with DJS may also have a liver that is sometimes enlarged and tender. The symptoms often do not present until puberty or adulthood. Factors that may worsen symptoms include: alcohol use, birth control pills, infection, and pregnancy. In most cases, treatment is not required.

ABCC2 gene. The ABCC2 gene codes for a protein called multidrug resistance protein 2 (MRP2). MRP2 transports substances out of cells. It is primarily found in the liver, but can also be found in the kidneys, intestine, and placenta during pregnancy.

Mutations in the ABCC2 gene affect the structure and function of the MRP2 protein. This may disrupt the body's ability to rid itself of bilirubin, resulting in a build up.