Elastin, the main component of arterial extracellular matrix, was thought
to have a purely structural role. Consistent with this view, elastin hemizygous
mice maintain arterial extensibility by increasing the number of elastic lamellae
during development. However, mice lacking elastin die of obstructive arterial pathology.
This pathology results from subendothelial proliferation and reorganization of
smooth muscle, cellular changes similar to those observed in atherosclerosis. Thus,
elastin is a molecular determinant of arterial morphogenesis and likely plays a
central role in vascular disease. Mice which are heterozygous and null for the
elastin gene have been developed. These mice are extremely useful for screening
for drugs useful for treating persons with atherosclerosis, hypertension, SVAS
or other vascular diseases.
