Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Morphologic features of bone in human osteopetrosis.

M H Helfrich1, D C Aronson, V Everts

  • 1Laboratory of Cell Biology and Histology, University of Leiden, The Netherlands.

Bone
|January 1, 1991
PubMed
Summary

Osteopetrosis is characterized by an excess of osteoclasts that fail to resorb bone, alongside a deficiency in bone-forming cells. A pathological calcified layer may contribute to the bone abnormalities observed in this condition.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Is hepatocellular carcinoma the same disease in children and adults? Comparison of histology, molecular background, and treatment in pediatric and adult patients.

Pediatric blood & cancer·2018
Same author

Aging does not change the compressive stiffness of mandibular condylar cartilage in horses.

Osteoarthritis and cartilage·2018
Same author

The intricate anatomy of the periodontal ligament and its development: Lessons for periodontal regeneration.

Journal of periodontal research·2017
Same author

Bone-site-specific responses to zoledronic acid.

Oral diseases·2016
Same author

Osteoblasts develop from isolated fetal mouse chondrocytes when co-cultured in high density with brain tissue.

In vitro cellular & developmental biology. Animal·2016
Same author

Increased epidermal thickness and abnormal epidermal differentiation in keloid scars.

The British journal of dermatology·2016

Area of Science:

  • Bone biology
  • Pathology
  • Cell biology

Background:

  • Osteopetrosis is a rare genetic disorder affecting bone remodeling.
  • Characterized by impaired osteoclast function and bone resorption.

Purpose of the Study:

  • To investigate the cellular and matrix abnormalities in osteopetrotic bone.
  • To elucidate the pathogenesis of malignant and benign osteopetrosis.

Main Methods:

  • Light and electron microscopy of trabecular bone biopsies.
  • Comparison of samples from patients with malignant osteopetrosis, benign osteopetrosis, and healthy controls.

Main Results:

  • Osteopetrotic osteoclasts exhibited minimal signs of bone resorption (absent ruffled borders and clear zones).

Related Experiment Videos

  • Malignant osteopetrosis cases showed significantly reduced osteoblasts, lining cells, and stromal cells.
  • A novel mineralized, acellular organic layer was observed on bone and cartilage matrices in six patients.

    • Conclusions:

    • Osteopetrotic osteoclasts are functionally impaired in bone resorption.
    • Deficiency in bone-forming cells and the presence of a pathological calcified layer contribute to osteopetrotic bone abnormalities.
    • These findings offer insights into the pathogenesis of osteopetrosis.