Search Results - "KIELTY, C. M"
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1
The molecular genetics of Marfan syndrome and related disorders
Published in Journal of Medical Genetics (01-10-2006)“…Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of connective tissue with prominent manifestations in the skeletal, ocular,…”
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2
Fibrillin microfibrils are reduced in skin exhibiting striae distensae
Published in British journal of dermatology (1951) (01-06-1998)“…Striae distensae (striae: stretch marks) are a common disfiguring condition associated with continuous and progressive stretching of the skin—as occurs during…”
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3
Fibrillin: from microfibril assembly to biomechanical function
Published in Philosophical transactions of the Royal Society of London. Series B. Biological sciences (28-02-2002)“…Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long–range…”
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Fibrillin-Rich Microfibrils are Reduced in Photoaged Skin. Distribution at the Dermal–Epidermal Junction
Published in Journal of investigative dermatology (01-05-1999)“…Chronic sun exposure results in photoaged skin with deep coarse wrinkles and loss of elasticity. We have examined the distribution and abundance of…”
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5
Mesenchymal Stromal Cells: Inhibiting PDGF Receptors or Depleting Fibronectin Induces Mesodermal Progenitors with Endothelial Potential
Published in Stem cells (Dayton, Ohio) (01-03-2014)“…Realizing the full therapeutic potential of mesenchymal stromal/stem cells (MSCs) awaits improved understanding of mechanisms controlling their fate. Using…”
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6
Fibrillin and the eye
Published in British journal of ophthalmology (01-11-2000)“…Fibrillin polymers form the structural scaffold of extensible microfibrils 1-3 which are present in ocular elastic tissues and are arranged in parallel bundles…”
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7
Distribution and expression of type VI collagen in photoaged skin
Published in British journal of dermatology (1951) (01-04-2001)“…Background Several of the characteristic clinical features of photoaged skin, including wrinkling, are thought to be dependent on changes in the dermal matrix…”
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Fibrillin-rich microfibrils: elastic biopolymers of the extracellular matrix
Published in Journal of muscle research and cell motility (01-01-2002)“…Fibrillin-rich microfibrils are evolutionarily ancient macromolecular assemblies of the extracellular matrix. They have unique extensible properties that endow…”
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Fibrillin-rich microfibrils of the extracellular matrix: ultrastructure and assembly
Published in Micron (Oxford, England : 1993) (01-02-2001)“…Fibrillin-rich microfibrils are a unique class of extensible connective tissue macromolecules. Their critical contribution to the establishment and maintenance…”
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10
Type VI Collagen Microfibrils: Evidence for a Structural Association with Hyaluronan
Published in The Journal of cell biology (01-08-1992)“…Type VI collagen, a widespread structural component of connective tissues, has been isolated in abundance from fetal bovine skin by a procedure involving…”
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11
Calcium Determines the Supramolecular Organization of Fibrillin-Rich Microfibrils
Published in The Journal of cell biology (04-05-1998)“…Microfibrils are ubiquitous fibrillin-rich polymers that are thought to provide long-range elasticity to extracellular matrices, including the zonular…”
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12
Age-related changes in the temporal and spatial distributions of fibrillin and elastin mRNAS and proteins in acute cutaneous wounds of healthy humans
Published in The Journal of pathology (01-09-1997)“…Elasticity and resilience of the skin are determined largely by the elastin framework, whose microfibrillar scaffold is composed of fibrillin. To date, the…”
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13
Clinical features of photodamaged human skin are associated with a reduction in collagen VII
Published in British journal of dermatology (1951) (01-09-1997)“…Summary Chronically sun‐exposed or photodamaged human skin is characterized by a number of clinical features, including wrinkles. However, little is known…”
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14
Catabolism of intact type VI collagen microfibrils: susceptibility to degradation by serine proteinases
Published in Biochemical and biophysical research communications (31-03-1993)“…We present the first direct biochemical evidence for the turnover of intact type VI collagen microfibrils. Matrix-degrading enzymes of the serine proteinase…”
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15
Keratinocytes express fibrillin and assemble microfibrils: implications for dermal matrix organization
Published in British journal of dermatology (1951) (01-07-1997)“…Summary Fibrillin–containing microfibrils are key architectural structures of the upper dermis and integral components of the dermal elastic fibre network…”
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16
Three-dimensional reconstructions of extracellular matrix polymers using automated electron tomography
Published in Journal of structural biology (01-04-2002)“…The extracellular matrix is an intricate network of macromolecules which provides support for cells and a framework for tissues. The detailed structure and…”
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17
Analyses of truncated fibrillin caused by a 366 bp deletion in the FBN1 gene resulting in Marfan syndrome
Published in Biochemical journal (15-09-1994)“…We studied fibrillin synthesis in cultured fibroblasts from 11 members of a three-generation family with Marfan syndrome, caused by a large in-frame deletion…”
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18
The supramolecular organisation of fibrillin-rich microfibrils determines the mechanical properties of bovine zonular filaments
Published in Journal of experimental biology (01-11-1999)“…The zonular filaments from the eyes of cows are rich in microfibrils containing fibrillin. Tensile tests, stress-relaxation tests and X-ray diffraction studies…”
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19
A novel hyaluronan-based biomaterial (Hyaff-11 ®) as a scaffold for endothelial cells in tissue engineered vascular grafts
Published in Biomaterials (01-12-2004)“…Current prosthetic small diameter vascular grafts show poor long-term patency rates, leading to the pursuit of a biological alternative. Hyaff-11 is a…”
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20
Microfibrillar elements in the synovial joint: presence of type VI collagen and fibrillin-containing microfibrils
Published in Annals of the rheumatic diseases (01-06-1993)“…OBJECTIVES--The aims were to isolate and positively identify the microfibrillar elements which have been observed in the synovial lining. In addition, synovial…”
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