Microenvironmental Regulation by Fibrillin-1

Mutations in the TGFβ Binding-Protein-Like Domain 5 of FBN1 Are Responsible for Acromicric and Geleophysic Dysplasias

(2011) Carine Le Goff et al.   AMERICAN JOURNAL OF HUMAN GENETICS

ADAMTS10 Protein Interacts with Fibrillin-1 and Promotes Its Deposition in Extracellular Matrix of Cultured Fibroblasts

(2011) Wendy E. Kutz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Latent TGF-β structure and activation

(2011) Minlong Shi et al.   NATURE

In VivoStudies of Mutant Fibrillin-1 Microfibrils

(2010) Noe L. Charbonneau et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prodomains of Transforming Growth Factor β (TGFβ) Superfamily Members Specify Different Functions

(2010) Gerhard Sengle et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in Fibrillin-1 Cause Congenital Scleroderma: Stiff Skin Syndrome

(2010) B. L. Loeys et al.   Science Translational Medicine

A Homozygous Mutation in ADAMTSL4 Causes Autosomal-Recessive Isolated Ectopia Lentis

(2009) Dina Ahram et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Homozygous Mutations in ADAMTS10 and ADAMTS17 Cause Lenticular Myopia, Ectopia Lentis, Glaucoma, Spherophakia, and Short Stature

(2009) Jose Morales et al.   AMERICAN JOURNAL OF HUMAN GENETICS

ADAMTSL-6 Is a Novel Extracellular Matrix Protein That Binds to Fibrillin-1 and Promotes Fibrillin-1 Fibril Formation

(2009) Ko Tsutsui et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Latent Transforming Growth Factor β-binding Proteins and Fibulins Compete for Fibrillin-1 and Exhibit Exquisite Specificities in Binding Sites

(2009) Robert N. Ono et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Targeting of Bone Morphogenetic Protein Growth Factor Complexes to Fibrillin

(2008) Gerhard Sengle et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ADAMTSL2 mutations in geleophysic dysplasia demonstrate a role for ADAMTS-like proteins in TGF-β bioavailability regulation

(2008) Carine Le Goff et al.   NATURE GENETICS