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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...
Bone Formation by Intramembranous Ossification01:29

Bone Formation by Intramembranous Ossification

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.
The process begins when mesenchymal cells in the embryonic skeleton gather together and differentiate into osteogenic cells, which then develop into...
Hormones and Bone Tissue01:17

Hormones and Bone Tissue

The endocrine system produces and secretes hormones, which interact with the skeletal system. These hormones control bone growth, maintain bone once it is formed, and remodel it.
Hormones That Influence Osteoblasts and/or Maintain the Matrix
Several hormones are necessary for controlling bone growth and maintaining the bone matrix. The pituitary gland secretes growth hormone (GH), which, as its name implies, controls bone growth. This happens in several ways: first, it triggers chondrocyte...
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 Remodeling and Repair01:31

Bone Remodeling and Repair

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 Formation by Endochondral Ossification01:24

Bone Formation by Endochondral Ossification

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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Related Experiment Video

Updated: Jul 11, 2026

Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification
07:23

Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification

Published on: December 3, 2016

Osteogenesis imperfecta:epidemiology and pathophysiology.

Elizabeth Martin1, Jay R Shapiro

  • 1The Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205, USA.

Current Osteoporosis Reports
|October 11, 2007
PubMed
Summary

Osteogenesis imperfecta (OI) is a genetic bone disorder affecting multiple systems. Research explores genetic mutations and evolving treatments like bisphosphonates and novel therapies.

Area of Science:

  • Genetics and Bone Biology
  • Connective Tissue Disorders

Background:

  • Osteogenesis imperfecta (OI) is the most common inherited connective tissue disorder, primarily affecting bone but with systemic manifestations.
  • OI classification has expanded beyond initial COL1A1/COL1A2 mutations to include genes like CRTAP and LEPRE1, particularly in recessive forms.
  • Systemic complications include ocular issues, dentinogenesis imperfecta, hearing loss, joint laxity, pulmonary disease, and short stature.

Purpose of the Study:

  • To review the genetic basis of Osteogenesis Imperfecta, including newly identified phenotypes and genes.
  • To discuss current challenges and considerations in the bisphosphonate treatment of OI.
  • To highlight emerging and future therapeutic strategies for OI.

Main Methods:

  • Literature review of genetic mutations associated with OI phenotypes.

More Related Videos

Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis
09:20

Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis

Published on: December 18, 2019

Related Experiment Videos

Last Updated: Jul 11, 2026

Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification
07:23

Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification

Published on: December 3, 2016

Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis
09:20

Laser Capture Microdissection of Mouse Embryonic Cartilage and Bone for Gene Expression Analysis

Published on: December 18, 2019

  • Analysis of treatment strategies, focusing on bisphosphonates and their limitations.
  • Exploration of novel and investigational therapies for OI.

    • Main Results:

    • OI is caused by mutations in collagen genes (COL1A1, COL1A2) and other genes involved in collagen modification (CRTAP, LEPRE1).
    • Bisphosphonate treatment for OI presents challenges in patient selection, outcome evaluation, and treatment duration, with differing responses in children versus adults.
    • New therapeutic avenues including PTH(1-34), Rank ligand inhibitors, and stem cell technology are under investigation.

    Conclusions:

    • Osteogenesis imperfecta is a complex systemic disorder with a growing understanding of its genetic underpinnings.
    • Current bisphosphonate treatments for OI require careful management and further research into optimal application.
    • Future research holds promise for more effective and targeted therapies for OI, including regenerative medicine approaches.