The molecular cloning and identification of mutations in ATP-binding casset
te transporters in hereditary diseases have greatly expanded our knowledge
of the normal physiology of intracellular lipid transport processes. In add
ition to the well-known ATP-binding cassette transporter A1 (ABCA1) molecul
e, ABC transporters belonging to the ABCG (NVhite) subfamily (ABCG1, ABCG5,
and ABCG8) have been shown to be critically involved in the regulation of
lipid-trafficking mechanisms in macrophages, hepatocytes, and intestinal mu
cosa cells. ABCG1, the product of a sterol-induced gene, participates in ch
olesterol and phospholipid efflux. The ABCG5 and ABCG8 transporters, defect
ive in beta -sitosterolemia, are also now considered interesting targets in
the control and influence of total body sterol homeostasis. In this review
, advances referring to the regulation and function of ABCG half-size trans
porters are summarized and discussed. In addition, new implications for the
transcriptional control, as well as the intracellular routing and localiza
tion, of these proteins are presented.