LTBPs in biology and medicine: LTBP diseases

The latent transforming growth factor (TGF) β binding proteins (LTBP) are crucial mediators of TGFβ function, as they control growth factor secretion, matrix deposition, presentation and activation. Deficiencies in specific LTBP isoforms yield discrete phenotypes representing defects in bone, lung a...

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Veröffentlicht in:	Matrix biology 2018-10, Vol.71-72, p.90-99
Hauptverfasser:	Rifkin, Daniel B., Rifkin, William J., Zilberberg, Lior
Format:	Artikel
Sprache:	eng
Schlagworte:	Aneurysm Animals Bone Diseases - metabolism Cardiovascular Diseases - metabolism Elastin maturation Extracellular Matrix - metabolism Fibrillin Genotype & phenotype Growth factors Humans Isoforms Latent TGF-beta Binding Proteins - deficiency Latent TGF-beta Binding Proteins - metabolism Latent TGFβ binding proteins Lung Diseases - metabolism Marfan syndrome Phenotypes Proteins Secretion Sensors Signal Transduction TGFβ TGFβ activation Transforming Growth Factor beta - metabolism
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