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Fibulins: a versatile family of extracellular matrix proteins

Nature reviews. Molecular cell biology, 2003-06, Vol.4 (6), p.479-489

2003

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COL6A3 Protein Deficiency in Mice Leads to Muscle and Tendon Defects Similar to Human Collagen VI Congenital Muscular Dystrophy

The Journal of biological chemistry, 2013-05, Vol.288 (20), p.14320-14331

2013

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Tumor-specific expression and alternative splicing of the COL6A3 gene in pancreatic cancer

Surgery, 2011-08, Vol.150 (2), p.306-315

2011

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Muscle Interstitial Fibroblasts Are the Main Source of Collagen VI Synthesis in Skeletal Muscle: Implications for Congenital Muscular Dystrophy Types Ullrich and Bethlem

Journal of neuropathology and experimental neurology, 2008-02, Vol.67 (2), p.144-154

2008

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Clinical Significance of Serum COL6A3 in Pancreatic Ductal Adenocarcinoma

Journal of gastrointestinal surgery, 2014, Vol.18 (1), p.7-15

2014

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Compound heterozygous mutations in fibulin-4 causing neonatal lethal pulmonary artery occlusion, aortic aneurysm, arachnodactyly, and mild cutis laxa

American journal of medical genetics. Part A, 2007-11, Vol.143A (22), p.2635-2641

2007

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Loss of fibulin-4 results in abnormal collagen fibril assembly in bone, caused by impaired lysyl oxidase processing and collagen cross-linking

Matrix biology, 2016-03, Vol.50, p.53-66

2016

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Fibulin-2 and fibulin-5 cooperatively function to form the internal elastic lamina and protect from vascular injury

Arteriosclerosis, thrombosis, and vascular biology, 2010-01, Vol.30 (1), p.68

2010

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Retinoic acid receptors are required for skeletal growth, matrix homeostasis and growth plate function in postnatal mouse

Developmental biology, 2009-04, Vol.328 (2), p.315-327

2009

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Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice

Cell and tissue research, 2016-06, Vol.364 (3), p.637-646

2016

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Loss of fibulin-2 protects against progressive ventricular dysfunction after myocardial infarction

Journal of molecular and cellular cardiology, 2012-01, Vol.52 (1), p.273-282

2012

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Ullrich Scleroatonic Muscular Dystrophy is Caused by Recessive Mutations in Collagen Type VI

Proceedings of the National Academy of Sciences - PNAS, 2001-06, Vol.98 (13), p.7516-7521

2001

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Fibulins in development and heritable disease

Birth defects research. Part C. Embryo today, 2004-03, Vol.72 (1), p.25-36

2004

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Fibulin-2 is essential for angiotensin II-induced myocardial fibrosis mediated by transforming growth factor (TGF)-β

Laboratory investigation, 2016-07, Vol.96 (7), p.773-783

2016

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New Molecular Mechanism for Ullrich Congenital Muscular Dystrophy: A Heterozygous In-Frame Deletion in the COL6A1 Gene Causes a Severe Phenotype

American journal of human genetics, 2003-08, Vol.73 (2), p.355-369

2003

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Fibulin-2 Is Dispensable for Mouse Development and Elastic Fiber Formation

Molecular and Cellular Biology, 2008-02, Vol.28 (3), p.1061-1067

2008

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

American journal of human genetics, 2003-04, Vol.72 (4), p.998-1004

2003

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Fibulin-2 deficiency attenuates angiotensin II-induced cardiac hypertrophy by reducing transforming growth factor-β signalling

Clinical science (1979), 2014-02, Vol.126 (4), p.275

2014

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Identification of Novel TGF-β/Smad Gene Targets in Dermal Fibroblasts using a Combined cDNA Microarray/Promoter Transactivation Approach

The Journal of biological chemistry, 2001-05, Vol.276 (20), p.17058-17062

2001

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Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy

Stem cell research & therapy, 2014-02, Vol.5 (1), p.21-21, Article 21

2014

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

The Journal of biological chemistry, 2009-06, Vol.284 (25), p.16872-16881

2009

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Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities

The Journal of biological chemistry, 2015-08, Vol.290 (35), p.21443-21459

2015

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Reduced Fibulin-2 Contributes to Loss of Basement Membrane Integrity and Skin Blistering in Mice Lacking Integrin α3β1 in the Epidermis

Journal of investigative dermatology, 2014-06, Vol.134 (6), p.1609-1617

2014

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Comparative Analysis of the Fibulin Protein Family: BIOCHEMICAL CHARACTERIZATION, BINDING INTERACTIONS, AND TISSUE LOCALIZATION

The Journal of biological chemistry, 2007-04, Vol.282 (16), p.11805-11816

2007

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Dysfunctional tendon collagen fibrillogenesis in collagen VI null mice

Matrix biology, 2011-01, Vol.30 (1), p.53-61

2011

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Fibulins: a versatile family of extracellular matrix proteins

COL6A3 Protein Deficiency in Mice Leads to Muscle and Tendon Defects Similar to Human Collagen VI Congenital Muscular Dystrophy

Tumor-specific expression and alternative splicing of the COL6A3 gene in pancreatic cancer

Muscle Interstitial Fibroblasts Are the Main Source of Collagen VI Synthesis in Skeletal Muscle: Implications for Congenital Muscular Dystrophy Types Ullrich and Bethlem

Clinical Significance of Serum COL6A3 in Pancreatic Ductal Adenocarcinoma

Compound heterozygous mutations in fibulin-4 causing neonatal lethal pulmonary artery occlusion, aortic aneurysm, arachnodactyly, and mild cutis laxa

Loss of fibulin-4 results in abnormal collagen fibril assembly in bone, caused by impaired lysyl oxidase processing and collagen cross-linking

Fibulin-2 and fibulin-5 cooperatively function to form the internal elastic lamina and protect from vascular injury

Retinoic acid receptors are required for skeletal growth, matrix homeostasis and growth plate function in postnatal mouse

Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice

Loss of fibulin-2 protects against progressive ventricular dysfunction after myocardial infarction

Ullrich Scleroatonic Muscular Dystrophy is Caused by Recessive Mutations in Collagen Type VI

Fibulins in development and heritable disease

Fibulin-2 is essential for angiotensin II-induced myocardial fibrosis mediated by transforming growth factor (TGF)-β

New Molecular Mechanism for Ullrich Congenital Muscular Dystrophy: A Heterozygous In-Frame Deletion in the COL6A1 Gene Causes a Severe Phenotype

Fibulin-2 Is Dispensable for Mouse Development and Elastic Fiber Formation

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

Fibulin-2 deficiency attenuates angiotensin II-induced cardiac hypertrophy by reducing transforming growth factor-β signalling

Identification of Novel TGF-β/Smad Gene Targets in Dermal Fibroblasts using a Combined cDNA Microarray/Promoter Transactivation Approach

Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy

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

Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities

Reduced Fibulin-2 Contributes to Loss of Basement Membrane Integrity and Skin Blistering in Mice Lacking Integrin α3β1 in the Epidermis

Comparative Analysis of the Fibulin Protein Family: BIOCHEMICAL CHARACTERIZATION, BINDING INTERACTIONS, AND TISSUE LOCALIZATION

Dysfunctional tendon collagen fibrillogenesis in collagen VI null mice

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