Wang, Q.

bulin fi bulin -4 tropoelastin mutation fi bres tsc 1 gene patient broblast culture elastin protein -fibre formation analysis expression figure genet fi broblasts medium antibody tropoelastin expression formation fi brillin tsc 2 gene -fibre signi virus fibulin tuberous sclerosis tsc patients sclerosis control result tuberou brillin signi ficant decrease decrease level wbs fi broblasts tsc 1 tropoelastin mrna pl 335 ficant cleveland cleveland clinic shrna deletion aorta change sequence immunostaining signi ficantly lerner research institute sample pl 337 family fibril elastogenesi study control plk viruses tropoelastin mrna level tsc 1 mutations department tissue fi bulin gapdh rt –pcr western-blot analysis disease author chromosome aortic amount journal research clinic extracellular erasmus mc skin fi broblasts syndrome foreskin fi broblasts