Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology

Making sense of latent TGFβ activation

2002 • 1,579 citations

Dysregulation of TGF-β activation contributes to pathogenesis in Marfan syndrome

2003 • 1,359 citations

Elastin is an essential determinant of arterial morphogenesis

1998 • 758 citations

Elastic fibres

2002 • 750 citations

Elastic fiber homeostasis requires lysyl oxidase–like 1 protein

2004 • 628 citations

Fibulin-5/DANCE is essential for elastogenesis in vivo

2002 • 600 citations

Fibulin-5 is an elastin-binding protein essential for elastic fibre development in vivo

2002 • 594 citations

Latent Transforming Growth Factor β-binding Protein 1 Interacts with Fibrillin and Is a Microfibril-associated Protein

2003 • 531 citations

Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1

1999 • 514 citations

Inactivation of the Lysyl Oxidase Gene Lox Leads to Aortic Aneurysms, Cardiovascular Dysfunction, and Perinatal Death in Mice

2002 • 465 citations

A critical role for elastin signaling in vascular morphogenesis and disease

2002 • 458 citations

The elastin gene is disrupted by a translocation associated with supravalvular aortic stenosis

1993 • 419 citations

Targetting of the gene encoding fibrillin–1 recapitulates the vascular aspect of Marfan syndrome

1997 • 385 citations

Novel arterial pathology in mice and humans hemizygous for elastin.

1998 • 351 citations

Phenotypic Alteration of Vascular Smooth Muscle Cells Precedes Elastolysis in a Mouse Model of Marfan Syndrome

2001 • 350 citations

Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome

2004 • 345 citations

Homozygosity for a missense mutation in fibulin-5 (FBLN5) results in a severe form of cutis laxa

2002 • 310 citations

Lysyl Oxidase Is Required for Vascular and Diaphragmatic Development in Mice

2003 • 294 citations

Regulation of limb patterning by extracellular microfibrils

2001 • 209 citations

Cell signaling events: a view from the matrix

2003 • 164 citations

Genetic Heterogeneity of Cutis Laxa: A Heterozygous Tandem Duplication within the Fibulin-5 (FBLN5) Gene

2003 • 152 citations

Fine tuning of growth factor signals depends on fibrillin microfibril networks

2004 • 121 citations

Scanning transmission electron microscopy mass analysis of fibrillin-containing microfibrils from foetal elastic tissues

1997 • 56 citations