Ontology highlight
ABSTRACT:
SUBMITTER: Van Laer L
PROVIDER: S-EPMC3722447 | biostudies-literature | 2013 Aug
REPOSITORIES: biostudies-literature
Van Laer Lut L Proost Dorien D Loeys Bart L BL
European journal of pediatrics 20120717 8
Heritable connective tissue diseases comprise a heterogeneous group of multisystemic disorders that are characterized by significant morbidity and mortality. These disorders do not merely result from defects in the amount or structure of one of the components of the extracellular matrix, as the extracellular matrix also serves other functions, including sequestration of cytokines, such as transforming growth factor beta (TGFβ). Indeed, disturbed TGFβ signaling was demonstrated in several heritab ...[more]