The inheritable causes of jaundice comprise a large group of conditions of
varying frequency, from Gilbert's syndrome which is relatively common, to t
he very rare Crigler-Najjar syndrome. Although these conditions have been w
ell characterized clinically and in some cases biochemically, the underlyin
g molecular defects were unknown because of a lack of knowledge about the p
rocess of bile secretion by hepatocytes. The recent cloning of several tran
sporters for bile acids and other organic anions has enabled a greater unde
rstanding of this process and allowed correlation of the malfunction of the
se genes with specific disease processes. This new knowledge will provide f
or precision in diagnosis, allow antenatal testing and provide opportunitie
s for gene therapy for some of the more serious disorders.