Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: Insight into the functional role of mutant fibrillin-1

Simple RNA enzymes with new and highly specific endoribonuclease activities

1988 • 1,118 citations

A small catalytic oligoribonucleotide

1987 • 938 citations

Regulation of connective tissue growth factor gene expression in human skin fibroblasts and during wound repair.

1993 • 706 citations

Regulation of PDGF and its receptors in fibrotic diseases

2004 • 697 citations

The Biology of the Small Leucine-rich Proteoglycans

1999 • 637 citations

Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans

2002 • 600 citations

The cadherin superfamily: diversity in form and function

2001 • 598 citations

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

2003 • 531 citations

Biological Catalysis by RNA

1986 • 496 citations

The CCN family: a new stimulus package

2003 • 495 citations

Fibrogenesis II. Metalloproteinases and their inhibitors in liver fibrosis

2000 • 463 citations

Fibromodulin-null Mice Have Abnormal Collagen Fibrils, Tissue Organization, and Altered Lumican Deposition in Tendon

1999 • 440 citations

CTGF and SMADs, Maintenance of Scleroderma Phenotype Is Independent of SMAD Signaling

2001 • 419 citations

Endothelin-1 Promotes Myofibroblast Induction through the ETA Receptor via a rac/Phosphoinositide 3-Kinase/Akt-dependent Pathway and Is Essential for the Enhanced Contractile Phenotype of Fibrotic Fibroblasts

2004 • 351 citations

A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation.

1996 • 290 citations

Tight-skin, a new mutation of the mouse causing excessive growth of connective tissue and skeleton.

1976 • 279 citations

Global Expression Profiling of Fibroblast Responses to Transforming Growth Factor-β1 Reveals the Induction of Inhibitor of Differentiation-1 and Provides Evidence of Smooth Muscle Cell Phenotypic Switching

2003 • 252 citations

Versican Interacts with Fibrillin-1 and Links Extracellular Microfibrils to Other Connective Tissue Networks

2002 • 212 citations

Ribozyme rescue of photoreceptor cells in P23H transgenic rats: Long-term survival and late-stage therapy

2000 • 203 citations

The Prodomain of BMP-7 Targets the BMP-7 Complex to the Extracellular Matrix

2005 • 201 citations

Mutations of FBN1 and genotype-phenotype correlations in Marfan syndrome and related fibrillinopathies

2002 • 192 citations

Cell Adhesion to Fibrillin-1 Molecules and Microfibrils Is Mediated by α5β1 and αvβ3 Integrins

2003 • 188 citations

Cell signaling events: a view from the matrix

2003 • 164 citations

Interactions of Fibrillin-1 with Heparin/Heparan Sulfate, Implications for Microfibrillar Assembly

2001 • 163 citations

Determinants in the N‐terminal domains of galectin‐3 for secretion by a novel pathway circumventing the endoplasmic reticulum–Golgi complex

1999 • 151 citations

Protein Interaction Studies of MAGP-1 with Tropoelastin and Fibrillin-1

2001 • 142 citations

Interaction of Tropoelastin with the Amino-terminal Domains of Fibrillin-1 and Fibrillin-2 Suggests a Role for the Fibrillins in Elastic Fiber Assembly

2000 • 132 citations

Large T antigens of simian virus 40 and polyomavirus efficiently establish primary fibroblasts

1986 • 131 citations

Structure of the Integrin Binding Fragment from Fibrillin-1 Gives New Insights into Microfibril Organization

2004 • 129 citations

Gene expression profiling reveals novel TGFβ targets in adult lung fibroblasts

2004 • 123 citations

Fine tuning of growth factor signals depends on fibrillin microfibril networks

2004 • 121 citations

Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology

2004 • 118 citations

The Tight Skin Mouse: Demonstration of Mutant Fibrillin-1 Production and Assembly into Abnormal Microfibrils

1998 • 116 citations

Mediation of Transforming Growth Factor-β1-Stimulated Matrix Contraction by Fibroblasts

2003 • 111 citations

Disrupting the IL-4 gene rescues mice homozygous for the tight-skin mutation from embryonic death and diminishes TGF-β production by fibroblasts

2002 • 107 citations

Microfibrils at Basement Membrane Zones Interact with Perlecan via Fibrillin-1

2005 • 106 citations

The C Terminus of Fragile X Mental Retardation Protein Interacts with the Multi-domain Ran-binding Protein in the Microtubule-organising Centre

2004 • 104 citations

Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies

1997 • 96 citations

New Insights into the Assembly of Extracellular Microfibrils from the Analysis of the Fibrillin 1 Mutation in the Tight skin Mouse

2000 • 92 citations

Hereditary Emphysema in the Tight-Skin Mouse

1984 • 90 citations

Enhanced expression of fibrillin-1, a constituent of the myocardial extracellular matrix in fibrosis

2005 • 88 citations

Increased Plasminogen Activator Inhibitor-1 in Keloid Fibroblasts May Account for their Elevated Collagen Accumulation in Fibrin Gel Cultures

2003 • 84 citations

Synthetic Hammerhead Ribozymes as Therapeutic Tools to Control Disease Genes

2005 • 84 citations

Monocyte chemoattractant protein 3 as a mediator of fibrosis: Overexpression in systemic sclerosis and the type 1 tight‐skin mouse

2003 • 78 citations

ILK is required for the assembly of matrix-forming adhesions and capillary morphogenesis in endothelial cells

2004 • 78 citations

Inhibition of fibrillin 1 expression using U1 snRNA as a vehicle for the presentation of antisense targeting sequence

1997 • 72 citations

Expression of latent TGF-beta binding proteins and association with TGF-beta1 and fibrillin-1 following arterial injury

2002 • 64 citations

Interleukin 4 and prolonged hypoxia induce a higher gene expression of lysyl hydroxylase 2 and an altered cross-link pattern: Important pathogenetic steps in early and late stage of systemic scleroderma?

2005 • 64 citations

Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril‐associated glycoprotein 2 and type I collagen

2004 • 55 citations

Molecular Structure and Interaction of Recombinant Human Type XVI Collagen

2004 • 55 citations

Hammerhead ribozymes selectively suppress mutant type I collagen mRNA in osteogenesis imperfecta fibroblasts

2000 • 55 citations

Analysis of the tight skin (Tsk1/+) mouse as a model for testing antifibrotic agents

2005 • 50 citations

Tissue-Specific Expression of an Anti-ras Ribozyme Inhibits Proliferation of Human Malignant Melanoma Cells

1996 • 50 citations

Congenital fascial dystrophy: Stiff skin syndrome—a human counterpart of the tight-skin mouse

1989 • 48 citations

Cleavage of collagen RNA transcripts by hammerhead ribozymes in vitro is mutation-specific and shows competitive binding effects

1997 • 45 citations

Collagenous abnormalities in the heart of the tight-skin mouse1

1987 • 45 citations

Activation of a fibroblast-specific enhancer of the Pro?2(I) collagen gene in tight-skin mice

2001 • 45 citations

Differential involvement of the integrin-linked kinase (ILK) in RhoA-dependent rearrangement of F-actin fibers and induction of connective tissue growth factor (CTGF)

2005 • 44 citations

Interaction of a novel cysteine and histidine‐rich cytoplasmic protein with galectin‐3 in a carbohydrate‐independent manner

2000 • 42 citations

Transforming growth factor-β1 inhibits tumor growth in a mouse melanoma model by down-regulating the plasminogen activation system

2003 • 41 citations

The tight-skin mouse: physical and biochemical properties of the skin.

1983 • 40 citations

Tight-Skin Mouse an Experimental Model for Scleroderma

1994 • 35 citations

Delivery of a hammerhead ribozyme specifically down-regulates the production of fibrillin-1 by cultured dermal fibroblasts

1996 • 31 citations

Fibulin-2 and Fibulin-5 Alterations in Tsk Mice Associated with Disorganized Hypodermal Elastic Fibers and Skin Tethering

2004 • 27 citations

Fibrillin 1 abnormalities in dermal fibroblast cultures from first-degree relatives of patients with systemic sclerosis (scleroderma)

2004 • 13 citations

Functional Properties and Molecular Characteristics of Autoantibodies Associated with Tight Skin Syndrome^a

1997 • 5 citations