Current topic
Disorders of cholesterol biosynthesis
Biochemistry Unit, Institute of
Child Health and Metabolic Unit, Great Ormond Street Hospital for
Children, London
Correspondence to: Dr Peter T Clayton, Biochemistry Unit, Institute of Child Health, 30 Guilford Street, London WC1N 1EH.
| The first 150 words of the full text of this article appear below. |
 |
Functions of cholesterol |
|---|
Sterols are important constituents of the cell membranes
of most eukaryotic cells. The cell membranes of terrestrial
vertebrates, including man, contain a single major sterol
species
cholesterol. Cholesterol is found particularly in external
cellular membranes (plasma membranes) and in the layers that make up
the myelin sheaths in the central and peripheral nervous systems. In
plasma membranes, the cholesterol molecules are intercalated between
the phospholipid molecules of each monolayer and reduce the movement of
their acyl chains (reduced "membrane fluidity"). Sterols also exert
a direct effect on proteins in the membrane. For example, the function of the human red cell hexose transporter is profoundly affected by the
content of cholesterol in the membrane and this cannot be related to
changes in fluidity.1 It has been discovered recently that
cholesterol has important interactions with proteins which control
embryonic developmentthe hedgehog proteins.2 During
biosynthesis, these proteins catalyse their own cleavage and
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