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Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis
Bone material strength is determined by several factors, such as bone mass, matrix composition, mineralization, architecture and shape. From a clinical perspective, bone fragility is classified as primary (i.e., genetic and rare) or secondary (i.e., acquired and common) osteoporosis. Understanding t...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7826666/ https://www.ncbi.nlm.nih.gov/pubmed/33435159 http://dx.doi.org/10.3390/ijms22020625 |
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author | El-Gazzar, Ahmed Högler, Wolfgang |
author_facet | El-Gazzar, Ahmed Högler, Wolfgang |
author_sort | El-Gazzar, Ahmed |
collection | PubMed |
description | Bone material strength is determined by several factors, such as bone mass, matrix composition, mineralization, architecture and shape. From a clinical perspective, bone fragility is classified as primary (i.e., genetic and rare) or secondary (i.e., acquired and common) osteoporosis. Understanding the mechanism of rare genetic bone fragility disorders not only advances medical knowledge on rare diseases, it may open doors for drug development for more common disorders (i.e., postmenopausal osteoporosis). In this review, we highlight the main disease mechanisms underlying the development of human bone fragility associated with low bone mass known to date. The pathways we focus on are type I collagen processing, WNT-signaling, TGF-ß signaling, the RANKL-RANK system and the osteocyte mechanosensing pathway. We demonstrate how the discovery of most of these pathways has led to targeted, pathway-specific treatments. |
format | Online Article Text |
id | pubmed-7826666 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-78266662021-01-25 Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis El-Gazzar, Ahmed Högler, Wolfgang Int J Mol Sci Review Bone material strength is determined by several factors, such as bone mass, matrix composition, mineralization, architecture and shape. From a clinical perspective, bone fragility is classified as primary (i.e., genetic and rare) or secondary (i.e., acquired and common) osteoporosis. Understanding the mechanism of rare genetic bone fragility disorders not only advances medical knowledge on rare diseases, it may open doors for drug development for more common disorders (i.e., postmenopausal osteoporosis). In this review, we highlight the main disease mechanisms underlying the development of human bone fragility associated with low bone mass known to date. The pathways we focus on are type I collagen processing, WNT-signaling, TGF-ß signaling, the RANKL-RANK system and the osteocyte mechanosensing pathway. We demonstrate how the discovery of most of these pathways has led to targeted, pathway-specific treatments. MDPI 2021-01-10 /pmc/articles/PMC7826666/ /pubmed/33435159 http://dx.doi.org/10.3390/ijms22020625 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review El-Gazzar, Ahmed Högler, Wolfgang Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title | Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title_full | Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title_fullStr | Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title_full_unstemmed | Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title_short | Mechanisms of Bone Fragility: From Osteogenesis Imperfecta to Secondary Osteoporosis |
title_sort | mechanisms of bone fragility: from osteogenesis imperfecta to secondary osteoporosis |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7826666/ https://www.ncbi.nlm.nih.gov/pubmed/33435159 http://dx.doi.org/10.3390/ijms22020625 |
work_keys_str_mv | AT elgazzarahmed mechanismsofbonefragilityfromosteogenesisimperfectatosecondaryosteoporosis AT hoglerwolfgang mechanismsofbonefragilityfromosteogenesisimperfectatosecondaryosteoporosis |