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Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
The Functions of the Skeletal System01:22

The Functions of the Skeletal System

The most apparent functions of the skeletal system are support, protection, and movement. However, bone tissue also performs several other critical metabolic functions. For one, the bone matrix acts as a reservoir for a number of minerals important to the functioning of the body, especially calcium and phosphorus. These minerals, present in the bone tissue, can be released back into the bloodstream when required. Calcium ions, for example, are essential for muscle contractions and controlling...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
What is the Skeletal System?01:02

What is the Skeletal System?

Overview
Osteoclasts in Bone Remodeling01:31

Osteoclasts in Bone Remodeling

Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during bone...
Bone Remodeling01:40

Bone Remodeling

Bone remodeling is a continuous and balanced process of bone resorption by osteoclasts and bone formation by osteoblasts. In adults, it helps maintain bone mass and calcium homeostasis. While mechanical stress can stimulate turnover as part of the normal maintenance and reparative process, several hormones also regulate bone remodeling.

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Related Experiment Video

Updated: Jul 6, 2026

Murine Hind Limb Long Bone Dissection and Bone Marrow Isolation
07:17

Murine Hind Limb Long Bone Dissection and Bone Marrow Isolation

Published on: April 14, 2016

Sclerosing bone disorders.

Marie-Christine de Vernejoul1

  • 1INSERM U606 and University Paris 7, Rheumatology Department, Hospital Lariboisière, Assistance Publique Hôpitaux de Paris, 2 rue Ambroise Paré, 75010 Paris, France. christine.devernejoul@lrb.aphp.fr

Best Practice & Research. Clinical Rheumatology
|March 11, 2008
PubMed
Summary

Hereditary sclerosing bone diseases, like osteopetrosis and Camurati-Engelman dysplasia, are diagnosed by distinct radiological signs. Understanding their genetic basis improves our knowledge of bone remodeling and potential osteoporosis treatments.

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A Novel in vivo Gene Transfer Technique and in vitro Cell Based Assays for the Study of Bone Loss in Musculoskeletal Disorders
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Last Updated: Jul 6, 2026

Murine Hind Limb Long Bone Dissection and Bone Marrow Isolation
07:17

Murine Hind Limb Long Bone Dissection and Bone Marrow Isolation

Published on: April 14, 2016

A Novel in vivo Gene Transfer Technique and in vitro Cell Based Assays for the Study of Bone Loss in Musculoskeletal Disorders
11:47

A Novel in vivo Gene Transfer Technique and in vitro Cell Based Assays for the Study of Bone Loss in Musculoskeletal Disorders

Published on: June 8, 2014

Area of Science:

  • Genetics
  • Orthopedics
  • Molecular Biology

Background:

  • Sclerosing bone disorders present diagnostic challenges, often identifiable through characteristic radiological features.
  • Hereditary osteocondensation arises from impaired bone resorption or increased bone formation, linked to specific gene mutations.

Purpose of the Study:

  • To review the molecular mechanisms underlying hereditary sclerosing bone disorders.
  • To highlight how understanding these disorders aids in comprehending bone remodeling processes.
  • To identify potential therapeutic targets for conditions like postmenopausal osteoporosis.

Main Methods:

  • Review of genetic mutations associated with various hereditary sclerosing bone disorders.
  • Analysis of radiological features linked to specific genetic defects.
  • Correlation of molecular pathways with bone remodeling and disease phenotypes.

Main Results:

  • Osteopetrosis subtypes are linked to genes affecting osteoclast function (e.g., RANK-L) and differentiation.
  • Albers-Schonberg disease involves mutations in chloride channel 7, impacting osteoclast acidification.
  • Camurati-Engelman dysplasia and other disorders result from mutations in TGF-beta, LRP5, and SOST genes, affecting bone formation and the Wnt pathway.

Conclusions:

  • Elucidation of molecular mechanisms in hereditary osteocondensation enhances understanding of bone remodeling.
  • These discoveries offer new therapeutic targets for postmenopausal osteoporosis, with some under clinical investigation.
  • Further research is needed to uncover the molecular basis of all hereditary osteocondensation disorders.