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1. Full Text Fibrillins
by Kumra, Heena and Reinhardt, Dieter P
Methods in Cell Biology, ISSN 0091-679X, 2018, Volume 143, pp. 223 - 246
Fibrillins are one of the major components of supramolecular fibrous structures in the extracellular matrix of elastic and nonelastic tissues, termed... 
Microfibrils | Extracellular matrix | Fibrillins | Elastic tissues | Fibrillinopathies | ORGANIZATION | HYBRID DOMAIN | MARFAN-SYNDROME | CALCIUM | DERMAL FIBROBLASTS | CELL-ADHESION | HEPARIN/HEPARAN SULFATE | MICROFIBRILS | BINDING | MUTATION DATABASE | CELL BIOLOGY | Fibrillins - chemistry | Molecular Imaging - instrumentation | Humans | Extracellular Matrix - chemistry | Recombinant Proteins - isolation & purification | Marfan Syndrome - etiology | Skin Diseases, Genetic - etiology | Microfibrils - chemistry | Recombinant Proteins - metabolism | Recombinant Proteins - ultrastructure | Contracture - pathology | Fibrillins - metabolism | Recombinant Proteins - chemistry | Skin Diseases, Genetic - pathology | Molecular Imaging - methods | Protein Interaction Mapping - instrumentation | Fibrillins - isolation & purification | Protein Interaction Mapping - methods | Extracellular Matrix - ultrastructure | Animals | Weill-Marchesani Syndrome - pathology | Marfan Syndrome - pathology | Fibrillins - ultrastructure | Extracellular Matrix - pathology | Weill-Marchesani Syndrome - etiology | Contracture - etiology
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2. Full Text Basic components of connective tissues and extracellular matrix: Elastin, fibrillin, fibulins, fibrinogen, fibronectin, laminin, tenascins and thrombospondins
by Halper, Jaroslava and Kjaer, Michael
Advances in Experimental Medicine and Biology, ISSN 0065-2598, 2014, Volume 802, pp. 31 - 47
Laminin | Elastin | Fibulins | Tenascins | Fibrillin
Conference Proceeding
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3. Full Text Elastic fibres
by Kielty, Cay M and Sherratt, Michael J and Shuttleworth, C. Adrian
Journal of Cell Science, ISSN 0021-9533, 07/2002, Volume 115, Issue 14, pp. 2817 - 2828
Elastic fibres are essential extracellular matrix macromolecules comprising an elastin core surrounded by a mantle of fibrillin-rich microfibrils. They endow... 
Microfibrils | Fibrillin | Elastin | Elastic fibres | MAGP | Fibulin | elastic fibres | BETA-BINDING-PROTEIN | EXTRACELLULAR-MATRIX MICROFIBRILS | X-RAY-DIFFRACTION | TIGHT-SKIN MOUSE | elastin | SUPRAMOLECULAR ORGANIZATION | microfibrils | CELL BIOLOGY | IN-VITRO | MARFAN-SYNDROME | FACTOR-LIKE DOMAINS | FIBRILLIN-RICH MICROFIBRILS | fibrillin | DEVELOPMENTAL EXPRESSION | fibulin | Elastin - metabolism | Elastin - ultrastructure | Elastic Tissue - ultrastructure | Humans | Extracellular Matrix - metabolism | Fibrillins | Elastic Tissue - metabolism | Extracellular Matrix - ultrastructure | Animals | Models, Animal | Microfilament Proteins - metabolism | Microfilament Proteins - ultrastructure | Molecular Structure | Microfibrils - metabolism | Microfibrils - ultrastructure | Protein Structure, Tertiary - physiology
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4. Full Text Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm
by Lindsay, M.E and Schepers, D and Bolar, N.A and Doyle, J.J and Gallo, E and Fert-Bober, J and Kempers, M.J.E and Fishman, E.K and Chen, Y and Myers, L and Bjeda, D and Oswald, G and Elias, A.F and Levy, H.P and Anderlid, B.M and Yang, M.H and Bongers, M.H.F and Timmermans, J and Braverman, A.C and Canham, N and Mortier, G.R and Brunner, H.G and Byers, P.H and Eyk, J. Van and Laer, L. van and Dietz, H.C and Loeys, B.L
Nature Genetics, ISSN 1061-4036, 2012, Volume 44, Issue 8, pp. 922 - 927
Loeys-Dietz syndrome (LDS) associates with a tissue signature for high transforming growth factor (TGF)-beta signaling but is often caused by heterozygous... 
PATHOGENESIS | GROWTH-FACTOR-BETA | ACTIVATION | FIBRILLIN | MARFAN-SYNDROME | MOUSE MODEL | GENETICS & HEREDITY | RECEPTOR | MICE | CONTRIBUTES | Aortic Aneurysm, Thoracic - genetics | Humans | Male | Aortic Aneurysm, Thoracic - pathology | Mice, Mutant Strains | Female | Microfilament Proteins - genetics | Disease Models, Animal | Fibrillin-1 | Signal Transduction | Fibrillins | Marfan Syndrome - genetics | Syndrome | Haploinsufficiency | Mice, Knockout | Loeys-Dietz Syndrome - pathology | Phenotype | Animals | Pedigree | Marfan Syndrome - pathology | Loeys-Dietz Syndrome - genetics | Mice | Mutation | Transforming Growth Factor beta2 - genetics | Transforming Growth Factor beta2 - deficiency | Gene mutations | Aneurysms | Physiological aspects | Genetic aspects | Research | Transforming growth factors | Health aspects | Risk factors | Proteins | Genotype & phenotype | Hypotheses | Cytokines | Collagen | Ligands | Experiments
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5. Full Text Mesenchymal-Stem-Cell-Induced Immunoregulation Involves FAS-Ligand-/FAS-Mediated T Cell Apoptosis
by Akiyama, Kentaro and Chen, Chider and Chen, WanJun and Wang, DanDan and Xu, Xingtian and Qu, Cunye and Yamaza, Takayoshi and Cai, Tao and Sun, Lingyun and Shi, Songtao
Cell Stem Cell, ISSN 1934-5909, 05/2012, Volume 10, Issue 5, pp. 544 - 555
Systemic infusion of bone marrow mesenchymal stem cells (BMMSCs) yields therapeutic benefit for a variety of autoimmune diseases, but the underlying mechanisms... 
RESPONSES | EXPERIMENTAL AUTOIMMUNE ENCEPHALOMYELITIS | GROWTH-FACTOR-BETA | ACTIVATION | PROTEIN | PATHWAY | CHEMOKINES | TOLERANCE | TISSUE | TRANSPLANTATION | CELL & TISSUE ENGINEERING | CELL BIOLOGY | T-Lymphocyte Subsets - immunology | Fas Ligand Protein - metabolism | Humans | fas Receptor - metabolism | Mesenchymal Stromal Cells - immunology | T-Lymphocytes, Regulatory - immunology | Scleroderma, Systemic - therapy | Colitis - therapy | Bone Marrow Cells - immunology | Colitis - chemically induced | Immunotherapy | Chemokine CCL2 - metabolism | Colitis - immunology | Microfilament Proteins - genetics | Transforming Growth Factor beta - immunology | Fibrillin-1 | Scleroderma, Systemic - genetics | Immune Tolerance | Fibrillins | Scleroderma, Systemic - immunology | Mice, Knockout | Animals | Mice | Dextran Sulfate - administration & dosage | Fas Ligand Protein - genetics | Transforming Growth Factor beta - metabolism | Mesenchymal Stem Cell Transplantation | Apoptosis | Fas antigen | Mesenchyme | CD95 antigen | Immunoregulation | Data processing | Lymphocytes T | Fibrillin | Macrophages | Immunological tolerance | CD4 antigen | FasL protein | Systemic sclerosis | Stem cells | Bone marrow | CD25 antigen | Foxp3 protein | Colitis | Autoimmune diseases | Monocyte chemoattractant protein 1
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6. Full Text 556 Advances in extracellular matrix proteomics through optimised sample preparation
by Eckersley, A.M and Watson, R.E and Knight, D and Griffiths, C.E and Sherratt, M.J
Journal of Investigative Dermatology, ISSN 0022-202X, 09/2016, Volume 136, Issue 9, pp. S255 - S255
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7. Full Text A new in vitro assay to test UVR protection of dermal extracellular matrix components by a flat spectrum sunscreen
by Hibbert, S.A and Costello, P and O'Connor, C and Bell, M and Griffiths, C.E.M and Watson, R.E.B and Sherratt, M.J
Journal of Photochemistry & Photobiology, B: Biology, ISSN 1011-1344, 10/2017, Volume 175, pp. 58 - 64
The efficacy of topical sunscreens is currently assessed by crude, costly and time consuming assays. We have previously demonstrated that components of the... 
PHOTOPROTECTION | VITAMIN-D | OXIDATIVE STRESS | BIOPHYSICS | SUN EXPOSURE | HUMAN SKIN | BIOCHEMISTRY & MOLECULAR BIOLOGY | SOLAR-RADIATION | FIBRILLIN-RICH MICROFIBRILS | ELASTIC TISSUES | PHOTOCARCINOGENESIS | DAMAGE | Microfibrils - chemistry | Dermis - metabolism | Fibrillins - chemistry | Electrophoresis, Polyacrylamide Gel | Humans | Extracellular Matrix - metabolism | Fibronectins - metabolism | Ultraviolet Rays | Fibronectins - chemistry | Protein Aggregates - radiation effects | Sunscreening Agents - chemistry | Microscopy, Atomic Force | Sunscreening Agents - pharmacology | Microfibrils - metabolism | Microfibrils - ultrastructure | Protein Aggregates - drug effects | Fibronectins | Atomic force microscopy | Toiletries industry | Sunscreens (Cosmetics) | Radiation
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8. Full Text Congenital contractural arachnodactyly complicated with aortic dilatation and dissection: Case report and review of literature
by Takeda, Norifumi and Morita, Hiroyuki and Fujita, Daishi and Inuzuka, Ryo and Taniguchi, Yuki and Imai, Yasushi and Hirata, Yasunobu and Komuro, Issei
American Journal of Medical Genetics Part A, ISSN 1552-4825, 10/2015, Volume 167, Issue 10, pp. 2382 - 2387
Congenital contractural arachnodactyly (CCA) is a connective tissue disease caused by mutations of the FBN2, which encodes fibrillin-2. CCA patients have a... 
congenital contractural arachnodactyly | splice mutation | aortic aneurysm | fibrillin‐2 | Beals syndrome | Congenital contractural arachnodactyly | Aortic aneurysm | Fibrillin-2 | Splice mutation | FBN2 MUTATIONS | fibrillin-2 | MARFAN-SYNDROME | GENETICS & HEREDITY | PHENOTYPE | FIBRILLIN GENE | PROBANDS | FAMILY | Dilatation, Pathologic - genetics | Exons | Humans | Middle Aged | Molecular Sequence Data | Arachnodactyly - complications | Male | Aorta - metabolism | Contracture - genetics | Carotid Artery, Internal, Dissection - complications | Contracture - complications | Dilatation, Pathologic - pathology | DNA Mutational Analysis | Base Sequence | Arachnodactyly - genetics | Female | Carotid Artery, Internal, Dissection - pathology | Child | Microfilament Proteins - genetics | Gene Expression | Contracture - pathology | Fibrillins | Genotype | Aorta - pathology | Carotid Artery, Internal, Dissection - genetics | Phenotype | Pedigree | Dilatation, Pathologic - complications | Heterozygote | Arachnodactyly - pathology | Mutation | Surgery | Genetic disorders | Marfan's syndrome | Congenital diseases | Splicing | Connective tissue diseases | Aorta | Arachnodactyly | Fibrillin | Dissection | Genetic screening
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9. Full Text Matrix-dependent perturbation of TGFβ signaling and disease
by Doyle, Jefferson J and Gerber, Elizabeth E and Dietz, Harry C
FEBS Letters, ISSN 0014-5793, 07/2012, Volume 586, Issue 14, pp. 2003 - 2015
Transforming growth factor beta (TGFβ) is a multipotent cytokine that is sequestered in the extracellular matrix (ECM) through interactions with a number of... 
Extracellular matrix | Integrin | Transforming growth factor beta | Fibrillin | Marfan syndrome | Stiff skin syndrome | HEREDITARY HEMORRHAGIC TELANGIECTASIA | RECEPTOR-TYPE-II | BIOCHEMISTRY & MOLECULAR BIOLOGY | ANGIOTENSIN-II | THORACIC AORTIC-ANEURYSM | SYSTEMIC-SCLEROSIS | WEILL-MARCHESANI-SYNDROME | CELL BIOLOGY | GROWTH-FACTOR-BETA | BIOPHYSICS | MARFAN-SYNDROME | MOUSE MODEL | SMOOTH-MUSCLE-CELLS | Cytokines - metabolism | Signal Transduction | Humans | Extracellular Matrix - metabolism | Homeostasis | Fibrillins | Integrins - metabolism | Syndrome | Phenotype | Animals | Marfan Syndrome - metabolism | Cell Membrane - metabolism | Mice | Microfilament Proteins - metabolism | Mutation | Transforming Growth Factor beta - metabolism | marfan syndrome | stiff skin syndrome | integrin | extracellular matrix | fibrillin
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10. Full Text Losartan, an AT1 Antagonist Prevents Aortic Aneurysm in a Mouse Model of Marfan Syndrome
by Jennifer P. Habashi and Daniel P. Judge and Tammy M. Holm and Ronald D. Cohn and Bart L. Loeys and Timothy K. Cooper and Loretha Myers and Erin C. Klein and Guosheng Liu and Carla Calvi and Megan Podowski and Enid R. Neptune and Marc K. Halushka and Djahida Bedja and Kathleen Gabrielson and Daniel B. Rifkin and Luca Carta and Francesco Ramirez and David L. Huso and Harry C. Dietz
Science, ISSN 0036-8075, 4/2006, Volume 312, Issue 5770, pp. 117 - 121
Aortic aneurysm and dissection are manifestations of Marfan syndrome (MFS), a disorder caused by mutations in the gene that encodes fibrillin-1. Selected... 
Airspace | Receptors | Architecture | Dilatation | Root growth | Placebos | Reports | Mice | Aortic aneurysm | Elastic tissue | Marfan syndrome | PATHOGENESIS | GROWTH-FACTOR-BETA | ACTIVATION | FIBRILLIN | MULTIDISCIPLINARY SCIENCES | RATS | RECEPTOR | SMOOTH-MUSCLE-CELLS | BLOCKADE | CONTRIBUTES | DILATATION | Angiotensin II Type 1 Receptor Blockers - therapeutic use | Propranolol - therapeutic use | Neutralization Tests | Aortic Aneurysm - etiology | Lung Diseases - drug therapy | Marfan Syndrome - drug therapy | Propranolol - administration & dosage | Antibodies - immunology | Transforming Growth Factor beta - antagonists & inhibitors | Female | Marfan Syndrome - complications | Microfilament Proteins - genetics | Adrenergic beta-Antagonists - administration & dosage | Adrenergic beta-Antagonists - therapeutic use | Angiotensin II Type 1 Receptor Blockers - administration & dosage | Aortic Aneurysm - prevention & control | Disease Models, Animal | Transforming Growth Factor beta - immunology | Fibrillin-1 | Lung - pathology | Pulmonary Alveoli - pathology | Signal Transduction | Fibrillins | Pregnancy Complications - drug therapy | Aorta - pathology | Pregnancy | Elastic Tissue - pathology | Animals | Receptor, Angiotensin, Type 1 - metabolism | Losartan - therapeutic use | Marfan Syndrome - metabolism | Marfan Syndrome - pathology | Losartan - administration & dosage | Lung Diseases - pathology | Mutation | Transforming Growth Factor beta - metabolism | Complications and side effects | Aortic aneurysms | Genetic aspects | Research | Risk factors
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11. Full Text Marfan's syndrome
by Judge, Daniel P and Dietz, Harry C
The Lancet, ISSN 0140-6736, 12/2005, Volume 366, Issue 9501, pp. 1965 - 1976
Marfan's syndrome is a systemic disorder of connective tissue caused by mutations in the extracellular matrix protein fibrillin 1. Cardinal manifestations... 
ROOT REPLACEMENT | MEDICINE, GENERAL & INTERNAL | PROSTHETIC HEART-VALVES | DURAL ECTASIA | MOLECULAR-WEIGHT HEPARIN | PECTUS-EXCAVATUM | THORACIC AORTIC-ANEURYSMS | PULMONARY-FUNCTION | CONNECTIVE-TISSUE | WEILL-MARCHESANI-SYNDROME | FETAL COMPLICATIONS | Diagnosis, Differential | Fibrillin-1 | Humans | Fibrillins | Male | Marfan Syndrome - physiopathology | Clinical Trials as Topic | Marfan Syndrome - genetics | Animals | Marfan Syndrome - drug therapy | Female | Mice | Microfilament Proteins - genetics | Adrenergic beta-Antagonists - therapeutic use | Complications and side effects | Development and progression | Care and treatment | Genetic aspects | Diagnosis | Marfan syndrome | Research | Analysis | Membrane proteins | Pathology | Medical diagnosis | Medical disorders | Medical treatment | Genomics | Marfan's syndrome | Animal models | Funding | Pathogenesis | Transforming growth factor-a | Aneurysms | Antagonists | Family medical history | Bone diseases | Fibrillin | High school basketball | Proteins | Connective tissues | Sinuses | Scoliosis | Surgery | Fingers & toes | Aorta | Extracellular matrix | Matrix protein | Dissection | Phenotypes | Life expectancy | Volleyball | Cytokines | Connective tissue diseases | Morbidity | Dislocations | Bone (long) | Mutation
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