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Skeletal dysplasias associated with mild myopathy-a clinical and molecular review.

Piróg KA, Briggs MD - J. Biomed. Biotechnol. (2010)

Bottom Line: Musculoskeletal system is a complex assembly of tissues which acts as scaffold for the body and enables locomotion.It is often overlooked that different components of this system may biomechanically interact and affect each other.The muscular complications seen in these cases are usually mild and may stem directly from the muscle defect and/or from the altered interactions between the individual components of the musculoskeletal system.

View Article: PubMed Central - PubMed

Affiliation: Wellcome Trust Centre for Cell Matrix Research, Faculty of Life Sciences, University of Manchester, Michael Smith Building, Oxford Road, Manchester M13 9PT, UK.

ABSTRACT
Musculoskeletal system is a complex assembly of tissues which acts as scaffold for the body and enables locomotion. It is often overlooked that different components of this system may biomechanically interact and affect each other. Skeletal dysplasias are diseases predominantly affecting the development of the osseous skeleton. However, in some cases skeletal dysplasia patients are referred to neuromuscular clinics prior to the correct skeletal diagnosis. The muscular complications seen in these cases are usually mild and may stem directly from the muscle defect and/or from the altered interactions between the individual components of the musculoskeletal system. A correct early diagnosis may enable better management of the patients and a better quality of life. This paper attempts to summarise the different components of the musculoskeletal system which are affected in skeletal dysplasias and lists several interesting examples of such diseases in order to enable better understanding of the complexity of human musculoskeletal system.

No MeSH data available.


Related in: MedlinePlus

(a) A schematic representation of processes that may influence bone formation and resorption [3]. (b) A graph illustrating the Hueter-Volkman law [4].
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Related In: Results  -  Collection


getmorefigures.php?uid=PMC2875749&req=5

fig1: (a) A schematic representation of processes that may influence bone formation and resorption [3]. (b) A graph illustrating the Hueter-Volkman law [4].

Mentions: Bone is a hard connective tissue consisting of osteocytes embedded in an abundant extracellular matrix (ECM). Bones act as a scaffold, attachment points, leverage, and protection for soft tissues. During life, and to enable growth, repair and mineralisation, the bone is constantly being remodelled by osteoblasts and osteoclasts [5]. Furthermore, bone is a dynamic tissue which is able to remodel in response to the mechanical load. For example, an increase in loading bone formation increases and resorption decreases whilst unloading of the bone has an opposite effect (Figure 1(a)) [3].


Skeletal dysplasias associated with mild myopathy-a clinical and molecular review.

Piróg KA, Briggs MD - J. Biomed. Biotechnol. (2010)

(a) A schematic representation of processes that may influence bone formation and resorption [3]. (b) A graph illustrating the Hueter-Volkman law [4].
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2875749&req=5

fig1: (a) A schematic representation of processes that may influence bone formation and resorption [3]. (b) A graph illustrating the Hueter-Volkman law [4].
Mentions: Bone is a hard connective tissue consisting of osteocytes embedded in an abundant extracellular matrix (ECM). Bones act as a scaffold, attachment points, leverage, and protection for soft tissues. During life, and to enable growth, repair and mineralisation, the bone is constantly being remodelled by osteoblasts and osteoclasts [5]. Furthermore, bone is a dynamic tissue which is able to remodel in response to the mechanical load. For example, an increase in loading bone formation increases and resorption decreases whilst unloading of the bone has an opposite effect (Figure 1(a)) [3].

Bottom Line: Musculoskeletal system is a complex assembly of tissues which acts as scaffold for the body and enables locomotion.It is often overlooked that different components of this system may biomechanically interact and affect each other.The muscular complications seen in these cases are usually mild and may stem directly from the muscle defect and/or from the altered interactions between the individual components of the musculoskeletal system.

View Article: PubMed Central - PubMed

Affiliation: Wellcome Trust Centre for Cell Matrix Research, Faculty of Life Sciences, University of Manchester, Michael Smith Building, Oxford Road, Manchester M13 9PT, UK.

ABSTRACT
Musculoskeletal system is a complex assembly of tissues which acts as scaffold for the body and enables locomotion. It is often overlooked that different components of this system may biomechanically interact and affect each other. Skeletal dysplasias are diseases predominantly affecting the development of the osseous skeleton. However, in some cases skeletal dysplasia patients are referred to neuromuscular clinics prior to the correct skeletal diagnosis. The muscular complications seen in these cases are usually mild and may stem directly from the muscle defect and/or from the altered interactions between the individual components of the musculoskeletal system. A correct early diagnosis may enable better management of the patients and a better quality of life. This paper attempts to summarise the different components of the musculoskeletal system which are affected in skeletal dysplasias and lists several interesting examples of such diseases in order to enable better understanding of the complexity of human musculoskeletal system.

No MeSH data available.


Related in: MedlinePlus