Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of
connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular …

Elastic fibers are extracellular components of higher vertebrates and confer elasticity and
resilience to numerous tissues and organs such as large blood vessels, lungs, and skin …

Cutis laxa is a rare disorder of elastic tissue resulting in loose, redundant, hypoelastic skin.
Both acquired and inherited forms exist, some of which have significant systemic …

Fibulins are a family of five extracellular matrix proteins characterized by tandem arrays of
epidermal growth factor-like domains and a C-terminal fibulin-type module. They are widely …

Abstract Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key
physiological functions: as supporting structures that impart tissue integrity and as regulators …

The fibulin family of extracellular matrix/matricellular proteins is composed of long fibulins
(fibulin-1,-2,-6) and short fibulins (fibulin-3,-4,-5,-7) and is involved in protein–protein …

Pelvic organ prolapse (POP) is a disabling disorder in women characterized by a loss of
pelvic floor support leading to the herniation of the uterus into or through the vagina. POP is …

Variants in the chromosomal region 10q26 are strongly associated with an increased risk for
age-related macular degeneration (AMD). Two potential AMD genes are located in this …

Interactions between the extracellular matrix (ECM) and cells are critical in embryonic
development, tissue homeostasis, physiological remodeling, and tumorigenesis …

Elastic fiber assembly requires deposition of elastin monomers onto microfibrils, the
mechanism of which is incompletely understood. Here we show that latent TGF-β binding …