Related Experiment Video
Updated: Jun 12, 2026
08:07
Three-Dimensional Bone Extracellular Matrix Model for Osteosarcoma
Published on: April 12, 2019
Shop talk: Sugars, bones, and a disease called multiple hereditary exostoses
Henry H Roehl1, Maurizio Pacifici
1MRC Centre for Developmental and Biomedical Genetics, University of Sheffield, Sheffield, UK. h.roehl@sheffield.ac.uk
Summary
Multiple hereditary exostoses (MHE) is an inherited condition causing bone tumors. Researchers convened to explore new diagnostic and treatment strategies for MHE, focusing on heparan sulfate synthesis.
Area of Science:
- Skeletal biology and genetics
- Biochemistry of glycosaminoglycans
Background:
- Multiple hereditary exostoses (MHE) is an autosomal dominant disorder.
- Characterized by the development of numerous osteochondromas (benign bone tumors).
- Associated with mutations in enzymes critical for heparan sulfate (HS) synthesis, increasing chondrosarcoma risk.
Framework:
- A 4-day meeting of researchers and physicians in Boston.
- Focused on generating novel ideas for MHE diagnosis, treatment, and cure.
- Discussions spanned clinical management, patient experiences, and fundamental biological mechanisms.
Implementation:
- Exploration of orthopedic surgical interventions.
- Sharing of patient personal experiences and perspectives.
- Delving into skeletal biology and the molecular role of HS.
Implications:
- Advancing understanding of MHE pathogenesis.
- Identifying potential therapeutic targets related to HS synthesis.
- Improving diagnostic and treatment paradigms for MHE patients.
Related Concept Videos
01:29Bone 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 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...
01:02What is the Skeletal System?
Overview
01:40Bone 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.
01:31Osteoclasts 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...
01:31Bone 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...
01:22The 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...