Open AccessJournal Article
Abnormal collagen fibril structure in the gravis form (type I) of Ehlers-Danlos syndrome.
Reads0
Chats0
TLDR
Altered abnormalities of fibrillogenesis are heterogeneous in origin; some might be due to primary defects in collagen whereas others may result from alterations of noncollagenous extracellular matrix components that influence collagen fibril formation.About:
This article is published in Laboratory Investigation.The article was published on 1979-02-01 and is currently open access. It has received 150 citations till now. The article focuses on the topics: Fibrillogenesis & Ehlers–Danlos syndrome.read more
Citations
More filters
Journal ArticleDOI
Ehlers‐Danlos syndromes: Revised nosology, Villefranche, 1997
TL;DR: This work proposes a revision of the classification of the Ehlers-Danlos syndromes based primarily on the cause of each type based on major and minor diagnostic criteria defined for each type and complemented whenever possible with laboratory findings.
Journal ArticleDOI
Mice that lack thrombospondin 2 display connective tissue abnormalities that are associated with disordered collagen fibrillogenesis, an increased vascular density, and a bleeding diathesis.
Themis R. Kyriakides,Yu Hong Zhu,Lynne T. Smith,Steven D. Bain,Zhantao Yang,Ming T. Lin,Keith G. Danielson,Renato V. Iozzo,Mary E. LaMarca,Cindy E. McKinney,Edward I. Ginns,Paul Bornstein +11 more
TL;DR: It seems likely that some of the diverse manifestations of this genetic disorder result from the ability of TSP2 to modulate the cell surface properties of mesenchymal cells, and thus, to affect cell functions such as adhesion and migration.
Journal ArticleDOI
A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency.
Joost Schalkwijk,Manon C. Zweers,Peter M. Steijlen,Willow B. Dean,Glen Taylor,Ivonne M.J.J. van Vlijmen,Brigitte van Haren,Walter L. Miller,James Bristow +8 more
TL;DR: This finding indicates that factors other than the collagens or collagen-processing enzymes can cause the Ehlers-Danlos syndrome and suggests a central role for tenascin-X in maintaining the integrity of collagenous matrix.
Journal ArticleDOI
Tenascin-X deficiency mimics Ehlers-Danlos syndrome in mice through alteration of collagen deposition.
Jau Ren Mao,Glen Taylor,Willow B. Dean,Willow B. Dean,Diane R. Wagner,Veena Afzal,Jeffrey C. Lotz,Edward M. Rubin,James Bristow,James Bristow +9 more
TL;DR: A causative role for TNXB is confirmed in human Ehlers–Danlos syndrome and it is suggested that tenascin-X is an essential regulator of collagen deposition by dermal fibroblasts.
Journal ArticleDOI
Using transmission electron microscopy and 3View to determine collagen fibril size and three-dimensional organization
Tobias Starborg,Nicholas S. Kalson,Nicholas S. Kalson,Yinhui Lu,Yinhui Lu,Alexander A. Mironov,Timothy F. Cootes,David F. Holmes,David F. Holmes,Karl E. Kadler,Karl E. Kadler +10 more
TL;DR: This work describes how to use 3View for studying collagen fibril organization in vivo and shows how to find and track individual fibrils.