Increased expression of Myosin binding protein H in the skeletal muscle of amyotrophic lateral sclerosis patients
Open accessAbstract
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.
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Activation of the RhoA/ROCK pathway has been implicated in the pathogenesis of ALS, although studied to a lesser degree and focusing on the disease-modifying effects of ROCK inhibition.
“Activation of the RhoA/ROCK pathway has also been implicated in the pathogenesis of ALS, although studied to a lesser degree and focusing on the disease-modifying effects of ROCK inhibition.”
From RhoA Signaling in Neurodegenerative Diseases (2022) · cites this paper as [212]
Section 3.4 reports higher ROCK2 expression in skeletal muscle from ALS patients, supporting an association between ROCK signaling and ALS. The Discussion does not establish increased ROCK activity or a pathogenic role for pathway activation, and the paper does not report disease-modifying effects of ROCK inhibition. Page numbers are unavailable in our copy. (LLM, conf=0.85)
Experimental — accuracy not yet measuredROCK expression was increased in the SOD1G93A transgenic mouse model of ALS.
“ROCK expression was increased in the SOD1G93A transgenic mouse model of ALS and in sporadic ALS patients [210,212].”
From RhoA Signaling in Neurodegenerative Diseases (2022) · cites this paper as [212]
Section 3.4 reports increased ROCK2 expression in skeletal muscle from ALS patients, and the Fig. 4 caption identifies them as sporadic ALS patients. The Discussion reports increased MyBP-H—not ROCK—in SOD1 G93A mice. Thus, this source does not establish increased ROCK expression in the mouse model. (LLM, conf=0.86)
Experimental — accuracy not yet measuredROCK expression was increased in sporadic ALS patients.
“ROCK expression was increased in the SOD1G93A transgenic mouse model of ALS and in sporadic ALS patients [210,212].”
From RhoA Signaling in Neurodegenerative Diseases (2022) · cites this paper as [212]
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Patterns of gene expression in atrophying skeletal muscles: response to food deprivation
Jagoe, R. T., Lecker, S. H., Gomes, M., & Goldberg, A. L. (2002) · The FASEB Journal · doi:10.1096/fj.02-0312com · Cited by 344 · see citing works
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RhoA Signaling in Neurodegenerative Diseases
Schmidt, S. I., Blaabjerg, M., Freude, K., & Meyer, M. (2022) · Cells · doi:10.3390/cells11091520 · Cited by 110 · see citing works
Proteomics Approaches for Biomarker and Drug Target Discovery in ALS and FTD
Hedl, T. J., San Gil, R., Cheng, F., Rayner, S. L., Davidson, J. M., De Luca, A., Villalva, M. D., Ecroyd, H., Walker, A. K., & Lee, A. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.00548 · Cited by 87 · see citing works
- Authors
- Conti, A., Riva, N., Pesca, M., Iannaccone, S., Cannistraci, C. V., Corbo, M., Previtali, S. C., Quattrini, A., & Alessio, M.
- Affiliations
- IRCCS Ospedale San Raffaele (IT)
- University of Salerno (IT)
- King Abdullah University of Science and Technology (SA)
- Corresponding author
- Alessio, Massimo
- ORCID
- Venue
- Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
- Publisher
- Elsevier BV
- Published
- 2014-01
- Volume
- 1842
- Issue
- 1
- Pages
- 99-106
- ISSN
- 0925-4439 (ISSN-L)
- OpenAlex
- W2079207986
- Cited by
- 68
- References
- 1
- Citations by year
- 2026: 5 · 2025: 6 · 2024: 10 · 2023: 5 · 2022: 6 · 2021: 6 · 2020: 5 · 2019: 5 · 2018: 5 · 2017: 7 · 2016: 4 · 2015: 2 · 2014: 2
- FWCI
- 1.56
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease (publishedVersion)
- PubMed (publishedVersion)
- Archivio Istituzionale della Ricerca - Università Vita-Salute San Raffaele (submittedVersion)
- King Abdullah University of Science and Technology Repository (King Abdullah University of Science and Technology) (submittedVersion)
- Funders
- MoH (RF07-ALS)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
- Keywords
- amyotrophic lateral sclerosis, skeletal muscle, differentially expressed proteins, biomarkers, actin-myosin interaction, cofilin-2, motor neuropathy, myopathy, ALS pathogenesis
- MeSH terms
- Adult, Aged, Amyotrophic Lateral Sclerosis, Cytoskeletal Proteins, Diagnosis, Differential, Female, Humans, Male, Middle Aged, Muscular Diseases, Myosins, Peripheral Nervous System Diseases, Protein Binding, Biomarkers, Gene Expression, Case-Control Studies, Muscle, Skeletal, Gene Expression Profiling, Proteomics, Cofilin 2, rho-Associated Kinases, Lim Kinases
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