Go to JCI Insight
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
  • Clinical Research and Public Health
  • Current issue
  • Past issues
  • By specialty
    • COVID-19
    • Cardiology
    • Gastroenterology
    • Immunology
    • Metabolism
    • Nephrology
    • Neuroscience
    • Oncology
    • Pulmonology
    • Vascular biology
    • All ...
  • Videos
    • Conversations with Giants in Medicine
    • Video Abstracts
  • Reviews
    • View all reviews ...
    • Complement Biology and Therapeutics (May 2025)
    • Evolving insights into MASLD and MASH pathogenesis and treatment (Apr 2025)
    • Microbiome in Health and Disease (Feb 2025)
    • Substance Use Disorders (Oct 2024)
    • Clonal Hematopoiesis (Oct 2024)
    • Sex Differences in Medicine (Sep 2024)
    • Vascular Malformations (Apr 2024)
    • View all review series ...
  • Viewpoint
  • Collections
    • In-Press Preview
    • Clinical Research and Public Health
    • Research Letters
    • Letters to the Editor
    • Editorials
    • Commentaries
    • Editor's notes
    • Reviews
    • Viewpoints
    • 100th anniversary
    • Top read articles

  • Current issue
  • Past issues
  • Specialties
  • Reviews
  • Review series
  • Conversations with Giants in Medicine
  • Video Abstracts
  • In-Press Preview
  • Clinical Research and Public Health
  • Research Letters
  • Letters to the Editor
  • Editorials
  • Commentaries
  • Editor's notes
  • Reviews
  • Viewpoints
  • 100th anniversary
  • Top read articles
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
Pseudoachondroplasia is caused through both intra- and extracellular pathogenic pathways
Robert Dinser, … , Mats Paulsson, Patrik Maurer
Robert Dinser, … , Mats Paulsson, Patrik Maurer
Published August 15, 2002
Citation Information: J Clin Invest. 2002;110(4):505-513. https://doi.org/10.1172/JCI14386.
View: Text | PDF
Article Genetics

Pseudoachondroplasia is caused through both intra- and extracellular pathogenic pathways

  • Text
  • PDF
Abstract

Research Article

Authors

Robert Dinser, Frank Zaucke, Florian Kreppel, Kjell Hultenby, Stefan Kochanek, Mats Paulsson, Patrik Maurer

×

Figure 4

Options: View larger image (or click on image) Download as PowerPoint
The matrix formed by cultures expressing mutant COMP is disturbed. Chond...
The matrix formed by cultures expressing mutant COMP is disturbed. Chondrocytes were fixed after 2 weeks of alginate culture following adenoviral transduction with 20 MOI (a) or 200 MOI (b–d). Electron microscopy of the pericellular matrix of cultures expressing low levels of mutant COMP reveals electron-dense amorphous aggregates (arrow) and some sparse collagen fibrils (a, arrowhead). The matrix formed after transduction with 200 MOI of the vector carrying the gene for mutant COMP has a similar structure, with amorphous aggregates (arrows) and thin collagen fibrils (arrowheads) lacking the regular proteoglycan stain (b). (c) Immunogold-labeling reveals a high concentration of COMP labeling in the amorphous aggregates compared with the low concentration next to the collagen fibers (arrowheads). (d) Chondrocytes overexpressing wild-type COMP form thick collagen fibers (arrow) carrying abundant electron-dense nodular structures (arrowheads). (e) The matrix formed by untransduced chondrocytes shows abundant thick collagen fibers (arrow) with occasional nodular structures corresponding to proteoglycans (arrowheads). Bars, 100 nm.

Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

Sign up for email alerts