Table 2.

Summary of similarities and differences between Wbs mice, Eln^+/− mice, SVAS patients, and WBS patients

	Mice				Human WBS	Human SVAS
	PDWbs	DDWbs	D/P Wbs	Eln+/−
Elastin gene expression	normal	reduced 38%	reduced 41%	reduced 40–50% [23]	reduced [16]	reduced [16]
Mean blood pressure	normal	increased by 18 mm Hg	increased by 9 mm Hg (non-significant)	increased by 16 mm Hg	increased in 13% of patients [16]	sometimes increased [41]
In vivo aortic strain	normal	reduced 6%	reduced 6%	reduced 5%	-	-
Number of MLUs	normal	normal	normal	increased 25–38%	increased [25]	increased [25, 41]
MLU structure	normal	fragmented, disorganized	fragmented, disorganized	thinner	thinner, fragmented [25]	thinner, fragmented [25, 41]
Aortic stenosis	no	no	no	no	often [16]	often [16]