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Related Concept Videos

Bone Disorders01:29

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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.
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All bones comprise an outer layer of compact bone, and an interior made up of spongy bone tissue, also called cancellous or trabecular bone. In long bones, spongy bone tissue is mainly found in the interior of the epiphyses (broad ends of the bone).
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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...
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Bone Formation by Intramembranous Ossification01:29

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Intramembranous ossification is one of the two processes involved in the development of bones within an embryo. The flat bones of the face, most of the cranial bones, and the clavicles are formed via this process. During intramembranous ossification, the bones develop directly from sheets of undifferentiated mesenchymal connective tissue.
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Compact Bone01:27

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Most bones contain compact and spongy osseous tissue, but their distribution and concentration vary based on the bone's overall function.
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Bone Formation by Endochondral Ossification01:24

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Bone formation, or ossification, begins around the sixth to seventh week of embryonic development. Most bones develop from a cartilaginous template through the process of endochondral ossification. Cartilage formation begins when clusters of mesenchymal cells differentiate into chondrocytes. These chondrocytes proliferate rapidly and secrete an extracellular matrix that becomes encased in a membrane called the perichondrium. The resulting cartilage model provides a template that resembles the...
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Updated: Feb 19, 2026

A Rat Tibial Growth Plate Injury Model to Characterize Repair Mechanisms and Evaluate Growth Plate Regeneration Strategies
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Juvenile Paget disease.

Stergios A Polyzos1, Tim Cundy2, Christos S Mantzoros3

  • 1First Department of Pharmacology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Metabolism: Clinical and Experimental
|October 30, 2017
PubMed
Summary
This summary is machine-generated.

Juvenile Paget disease (JPD) is a rare genetic disorder causing rapid bone turnover due to TNFRSF11B gene mutations. Early bisphosphonate treatment can improve skeletal deformities, but lifelong therapy may be needed.

Keywords:
Alkaline phosphataseDenosumabJuvenile Paget diseaseOsteoprotegerinReceptor activator of nuclear factor-κB

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Area of Science:

  • Genetics
  • Bone Biology
  • Rare Diseases

Background:

  • Juvenile Paget disease (JPD) is a rare genetic disorder characterized by extremely rapid bone turnover.
  • It is primarily caused by mutations in the TNFRSF11B gene, which encodes osteoprotegerin (OPG).
  • The loss of OPG function leads to severe skeletal and extra-skeletal manifestations, including deformities, hearing loss, and vascular issues.

Purpose of the Study:

  • To summarize the understanding of Juvenile Paget disease (JPD) pathophysiology, clinical features, and treatment strategies.
  • To highlight the role of TNFRSF11B gene mutations and osteoprotegerin (OPG) deficiency.
  • To discuss current and potential therapeutic interventions for JPD.

Main Methods:

  • Review of existing literature on Juvenile Paget disease (JPD).
  • Analysis of clinical manifestations and biochemical markers associated with OPG deficiency.
  • Evaluation of treatment outcomes with bisphosphonates and other agents.

Main Results:

  • JPD presents with progressive skeletal deformity in childhood and extra-skeletal complications.
  • Biochemical hallmarks include very high alkaline phosphatase and bone turnover markers.
  • Bisphosphonates are effective in reducing bone turnover and ameliorating skeletal phenotypes when initiated early.
  • Limited data suggest favorable outcomes with recombinant OPG and denosumab.

Conclusions:

  • Juvenile Paget disease (JPD) requires early and potentially lifelong treatment with anti-resorptive agents like bisphosphonates to manage rapid bone turnover and skeletal deformities.
  • Recombinant OPG offers a potential replacement therapy but is not clinically available.
  • Establishing an international patient registry is recommended for improved follow-up and research.