The interest of mammalian biologists in ATP-binding cassette (ABC) pro
teins is relatively recent. However, ABC proteins are widespread in di
stribution and have long been known to play an important transport rol
e in prokaryotes. This review includes a brief overview of the structu
re, regulation, and varied functions of ABC proteins in different cell
types as well as a synopsis of the emerging role of ABC proteins in h
uman biology and disease. The review then focuses on the established (
canalicular secretion of organic cations by the multidrug resistance,
or MDR 1, gene product; ductular secretion of fluid and electrolytes m
ediated by CFTR), probable (biliary phospholipid secretion by the MDR
2 gene product; secretion of non bile acid organic anions by the multi
drug resistance protein, or MRP) and possible (bile acid secretion; bi
liary secretion of the signaling molecule, ATP; hormone transport) rol
es of known and novel ABC proteins in hepatobiliary secretion.