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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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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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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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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

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

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

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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