Increased expression of Myosin binding protein H in the skeletal muscle of amyotrophic lateral sclerosis patients

Abstract
Amyotrophic lateral sclerosis (ALS) is a severe and fatal neurodegenerative disease of still unknown pathogenesis. Recent findings suggest that the skeletal muscle may play an active pathogenetic role. To investigate ALS's pathogenesis and to seek diagnostic markers, we analyzed skeletal muscle biopsies with the differential expression proteomic approach. We studied skeletal muscle biopsies from healthy controls (CN), sporadic ALS (sALS), motor neuropathies (MN) and myopathies (M). Pre-eminently among several differentially expressed proteins, Myosin binding protein H (MyBP-H) expression in ALS samples was anomalously high. MyBP-H is a component of the thick filaments of the skeletal muscle and has strong affinity for myosin, but its function is still unclear. High MyBP-H expression level was associated with abnormal expression of Rho kinase 2 (ROCK2), LIM domain kinase 1 (LIMK1) and cofilin2, that might affect the actin-myosin interaction. We propose that MyBP-H expression level serves, as a putative biomarker in the skeletal muscle, to discriminate ALS from motor neuropathies, and that it signals the onset of dysregulation in actin-myosin interaction; this in turn might contribute to the pathogenesis of ALS. © 2013 Elsevier B.V.

Citation
Conti, A., Riva, N., Pesca, M., Iannaccone, S., Cannistraci, C. V., Corbo, M., … Alessio, M. (2014). Increased expression of Myosin binding protein H in the skeletal muscle of amyotrophic lateral sclerosis patients. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1842(1), 99–106. doi:10.1016/j.bbadis.2013.10.013

Acknowledgements
This work was supported by MoH, RF07-ALS. The authors declare no conflict of interest

Publisher
Elsevier BV

Journal
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

DOI
10.1016/j.bbadis.2013.10.013

PubMed ID
24184715

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