This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Hereditary Hyperbilirubinemia & Jaundice, Subject name: Medicine
MRP2 transpoer defect leads to development of? 
Select and explain. 
A. Menke's disease, B. Dubin Johnson syndrome, C. Familial intrahepatic cholestasis, D. Benign recurrent intrahepatic cholestasis
B: Dubin Johnson syndrome
Answer: B: Dubin Johnson syndrome 
Explanation: MRP2 defect: Multidrug resistance-associated protein 2 (MRP2), an ATP-dependent canalicular membrane transpoer. It is defective in Dubin Johnson syndrome. This protein is encoded by gene known as ABCC2 Gene which is mutated. Dubin Johnson syndrome- It is an autosomal recessive disorder occurs due to defect in MRP-2 protein. kernicterus is absent , with liver biopsy showing black pigmentation. Mild jaundice is seen with normal bile acids level.