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Are mechanically sensitive regulators involved in the function and (patho)physiology of cerebral palsy-related contractures?

Abstract：

:Skeletal muscle tissue is mechanosensitive, as it is able to sense mechanical impacts and to translate these into biochemical signals making the tissue adapt. Among its mechanosensitive nature, skeletal muscle tissue is the largest metabolic organ of the human body. Disturbances in skeletal muscle mechanosensing and metabolism cause and contribute to many diseases, i.e. muscular dystrophies/myopathies, cardiovascular diseases, COPD or diabetes mellitus type 2. A less commonly focused muscle-related disorder is clinically known as muscle contractures that derive from cerebral palsy (CP) conditions in young and adults. Muscle contractures are characterized by gradually increasing passive muscle stiffness resulting in complete fixation of joints. Different mechanisms have been identified in CP-related contractures, i.e. altered calcium handling, altered metabolism or altered titin regulation. The muscle-related extracellular matrix (ECM), specifically collagens, plays a role in CP-related contractures. Herein, we focus on mechanically sensitive complexes, known as costameres (Cstms), and discuss their potential role in CP-related contractures. We extend our discussion to the ECM due to the limited knowledge of its role in CP-related contractures. The aims of this review are (1) to summarize CP-related contracture mechanisms, (2) to raise novel hypotheses on the genesis of contractures with a focus on Cstms, and (3) to stimulate novel approaches to study CP-related contractures.

journal_name

J Muscle Res Cell Motil

journal_title

Journal of muscle research and cell motility

authors

Pingel J,Suhr F

doi

10.1007/s10974-017-9489-1

subject

Has Abstract

pub_date

2017-08-01 00:00:00

pages

317-330

issue

3-4

eissn

0142-4319

issn

1573-2657

pii

10.1007/s10974-017-9489-1

journal_volume

38

pub_type

杂志文章,评审

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