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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 tendon hierarchical structure and a transmission electron microscopy image of a tenocyte embedded in the collagen matrix. (b) A histological (Gomori trichrome, staining collagenous tissues blue) image of a longitudinal section of patellar tendon showing parallel running collagen bundles in the tissue.
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fig3: (a) A schematic representation of tendon hierarchical structure and a transmission electron microscopy image of a tenocyte embedded in the collagen matrix. (b) A histological (Gomori trichrome, staining collagenous tissues blue) image of a longitudinal section of patellar tendon showing parallel running collagen bundles in the tissue.

Mentions: Tendon (Figures 3(a) and 3(b)) and ligament are soft collagenous tissues, linking muscle to bone and bone to bone, respectively. They consists of densely packed bundles of fibrous collagen and are characterised by high tensile strength and elasticity [5]. Tendon and ligament both have similar hierarchical structure, consisting of fascicles, which are built of smaller basic fibrils and fibroblasts [36]. The main component of the basic fibril is type I collagen. Both ligaments and tendons are surrounded by a sheath of transparent material called the synovial membrane, a delicate connective tissue layer secreting synovium, a viscous lubricating fluid [5].


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

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

(a) A schematic representation of tendon hierarchical structure and a transmission electron microscopy image of a tenocyte embedded in the collagen matrix. (b) A histological (Gomori trichrome, staining collagenous tissues blue) image of a longitudinal section of patellar tendon showing parallel running collagen bundles in the tissue.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig3: (a) A schematic representation of tendon hierarchical structure and a transmission electron microscopy image of a tenocyte embedded in the collagen matrix. (b) A histological (Gomori trichrome, staining collagenous tissues blue) image of a longitudinal section of patellar tendon showing parallel running collagen bundles in the tissue.
Mentions: Tendon (Figures 3(a) and 3(b)) and ligament are soft collagenous tissues, linking muscle to bone and bone to bone, respectively. They consists of densely packed bundles of fibrous collagen and are characterised by high tensile strength and elasticity [5]. Tendon and ligament both have similar hierarchical structure, consisting of fascicles, which are built of smaller basic fibrils and fibroblasts [36]. The main component of the basic fibril is type I collagen. Both ligaments and tendons are surrounded by a sheath of transparent material called the synovial membrane, a delicate connective tissue layer secreting synovium, a viscous lubricating fluid [5].

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