[HTML][HTML] Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences

NL Charbonneau, BJ Dzamba, RN Ono… - Journal of Biological …, 2003 - ASBMB
Fibrillins are microfibril-forming extracellular matrix macromolecules that modulate skeletal
development. In humans, mutations in fibrillins result in long bone overgrowth as well as
other distinct phenotypes. Whether fibrillins form independent microfibrillar networks or can
co-polymerize, forming a single microfibril, is not known. However, this knowledge is
required to determine whether phenotypes arise because of loss of singular or composite
functions of fibrillins. Immunolocalization experiments using tissues and de novo matrices …