(Q40429554)

20 September 2017

1 reference

title

Fibrillin-containing microfibrils: structure and function in health and disease (English)

1 reference

20 September 2017

Fibrillin-containing microfibrils: Structure and function in health and disease (English)

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

24 May 2024

author name string

C. M. Kielty

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A7584608

24 May 2024

C. A. Shuttleworth

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A7584608

24 May 2024

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=7584608

24 May 2024

publication date

1 August 1995

1 reference

full work available at URL

20 September 2017

https://api.elsevier.com/content/article/PII:135727259500028N?httpAccept=text/xml

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

https://api.elsevier.com/content/article/PII:135727259500028N?httpAccept=text/plain

24 May 2024

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

International Journal of Biochemistry & Cell Biology

24 May 2024

published in

1 reference

20 September 2017

volume

27

1 reference

20 September 2017

issue

8

1 reference

20 September 2017

page(s)

747-760

1 reference

Missense mutations impair intracellular processing of fibrillin and microfibril assembly in Marfan syndrome

20 September 2017

cites work

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1 reference

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24 May 2024

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Type VI collagen in extracellular, 100-nm periodic filaments and fibrils: identification by immunoelectron microscopy

24 May 2024

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Elastin-Associated Microfibrils and Microfibrillar Proteins

24 May 2024

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A second locus for Marfan syndrome maps to chromosome 3p24.2-p25.

24 May 2024

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Fibrillin binds calcium and is coded by cDNAs that reveal a multidomain structure and alternatively spliced exons at the 5' end

24 May 2024

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Fibrillin Immunoreactive Fibers Constitute a Unique Network in the Human Dermis: Immunohistochemical Comparison of the Distributions of Fibrillin, Vitronectin, Amyloid P Component, and Orcein Stainable Structures in Normal Skin and Elastosis

24 May 2024

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Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene

24 May 2024

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Marfan phenotype variability in a family segregating a missense mutation in the epidermal growth factor-like motif of the fibrillin gene

24 May 2024

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Clustering of fibrillin (FBN1) missense mutations in Marfan syndrome patients at cysteine residues in EGF-like domains

24 May 2024

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The skipping of constitutive exons in vivo induced by nonsense mutations

24 May 2024

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Four novel FBN1 mutations: significance for mutant transcript level and EGF-like domain calcium binding in the pathogenesis of Marfan syndrome

24 May 2024

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Rotary shadowing of collagen monomers, oligomers, and fibrils during tendon fibrillogenesis

24 May 2024

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Key residues involved in calcium-binding motifs in EGF-like domains

24 May 2024

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Identification of a novel nonsense mutation in the fibrillin gene (FBN1) using nonisotopic techniques

24 May 2024

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A novel fibrillin mutation in the Marfan syndrome which could disrupt calcium binding of the epidermal growth factor-like module

24 May 2024

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67-kD elastin-binding protein is a protective "companion" of extracellular insoluble elastin and intracellular tropoelastin

24 May 2024

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Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome

24 May 2024

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Ultrastructural immunolocalization of lysyl oxidase in vascular connective tissue

24 May 2024

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Location on chromosome 15 of the gene defect causing Marfan syndrome

24 May 2024

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Mutations in the fibrillin gene responsible for dominant ectopia lentis and neonatal Marfan syndrome

24 May 2024

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Anti-elastofibril monoclonal antibody NKH-1: production and application

24 May 2024

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Extraction of extendable beaded structures and their identification as fibrillin-containing extracellular matrix microfibrils

24 May 2024

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1 reference

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Preparation of a specific antiserum towards the microfibrillar protein of elastic tissues

24 May 2024

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Isolation and ultrastructural analysis of microfibrillar structures from foetal bovine elastic tissues. Relative abundance and supramolecular architecture of type VI collagen assemblies and fibrillin

24 May 2024

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Attachment of human vascular smooth muscles cells to intact microfibrillar assemblies of collagen VI and fibrillin

24 May 2024

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Fibrillin secretion and microfibril assembly by Marfan dermal fibroblasts

24 May 2024

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Catabolism of intact fibrillin microfibrils by neutrophil elastase, chymotrypsin and trypsin.

24 May 2024

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The role of calcium in the organization of fibrillin microfibrils

24 May 2024

45

1 reference

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Abnormal fibrillin assembly by dermal fibroblasts from two patients with Marfan syndrome

24 May 2024

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Microfibrillar elements of the dermal matrix

24 May 2024

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Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes

24 May 2024

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Microfibrils: Fine filamentous components of the tissue space

24 May 2024

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Ligand requirements for Ca2+ binding to EGF-like domains

24 May 2024

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24 May 2024

The elastic fiber

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1 reference

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Marfan syndrome: defective synthesis, secretion, and extracellular matrix formation of fibrillin by cultured dermal fibroblasts

24 May 2024

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1 reference

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Genomic organization of the sequence coding for fibrillin, the defective gene product in Marfan syndrome.

24 May 2024

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Decreased extracellular deposition of fibrillin and decorin in neonatal Marfan syndrome fibroblasts

24 May 2024

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Analyses of truncated fibrillin caused by a 366 bp deletion in the FBN1 gene resulting in Marfan syndrome

24 May 2024

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The fibrillin-Marfan syndrome connection

24 May 2024

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The elastic fiber. I. The separation and partial characterization of its macromolecular components

24 May 2024

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Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils

24 May 2024

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Changes in the components of extracellular matrix and in growth properties of cultured aortic smooth muscle cells upon ascorbate feeding

24 May 2024

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Extracellular matrix microfibrils are composed of core proteins coated with fibronectin

24 May 2024

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The identification of glycoproteins associated with elastic-tissue microfibrils

24 May 2024

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The nature of the microfibrillar glycoproteins of elastic fibres. A biosynthetic study

24 May 2024

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Identification of extractable proteins from the bovine ocular zonule: major zonular antigens of 32kD and 250kD.

24 May 2024

68

1 reference

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Rotary shadowing of collagen monomers, oligomers, and fibrils during tendon fibrillogenesis

24 May 2024

70

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

Mutation screening of complete fibrillin-1 coding sequence: report of five new mutations, including two in 8-cysteine domains

24 May 2024

71

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

Immunization with undenatured bovine zonular fibrils results in monoclonal antibodies to fibrillin

24 May 2024

72

1 reference

https://api.crossref.org/works/10.1016/1357-2725(95)00028-N

Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices

24 May 2024

73

1 reference

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24 May 2024

Identifiers

1 reference

20 September 2017

1 reference