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Myofiber type shift...
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Behzadi, ArvinUmeå universitet,Institutionen för integrativ medicinsk biologi (IMB),Neurovetenskaper
(author)
Myofiber type shift in extraocular muscles in amyotrophic lateral sclerosis
- Article/chapterEnglish2023
Publisher, publication year, extent ...
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Association for Research in Vision and Ophthalmology (ARVO),2023
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electronicrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:umu-210285
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https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-210285URI
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https://doi.org/10.1167/iovs.64.5.15DOI
Supplementary language notes
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Language:English
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Summary in:English
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Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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Purpose: To investigate changes in myofiber composition in the global layer (GL) and orbital layer (OL) of extraocular muscles (EOMs) from terminal amyotrophic lateral sclerosis (ALS) donors.Methods: Medial recti muscles collected postmortem from spinal-onset ALS, bulbar-onset ALS, and healthy control donors were processed for immunofluorescence with antibodies against myosin heavy chain (MyHC) IIa, MyHCI, MyHCeom, laminin, neurofilaments, synaptophysin, acetylcholine receptor γ-subunit, and α-bungarotoxin.Results: The proportion of myofibers containing MyHCIIa was significantly smaller and MyHCeom was significantly larger in the GL of spinal-onset ALS and bulbar-onset ALS donors compared to control donors. Changes in the GL were more prominent in the bulbar-onset ALS donors, with a significantly larger proportion of myofibers containing MyHCeom being present compared to spinal-onset ALS donors. There were no significant differences in the myofiber composition in the OL. In the spinal-onset ALS donors, the proportions of myofibers containing MyHCIIa in the GL and MyHCeom in the OL were significantly correlated with the disease duration. Neurofilament and synaptophysin were present at motor endplates of myofibers containing MyHCeom in ALS donors.Conclusions: The EOMs of terminal ALS donors displayed changes in the fast-type myofiber composition in the GL, with a more pronounced alteration in bulbar-onset ALS donors. Our results align with the worse prognosis and subclinical changes in eye movement function previously observed in bulbar-onset ALS patients and suggest that the myofibers in the OL might be more resistant to the pathological process in ALS.
Subject headings and genre
Added entries (persons, corporate bodies, meetings, titles ...)
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Tjust, Anton ErikUmeå universitet,Institutionen för integrativ medicinsk biologi (IMB),Neurovetenskaper(Swepub:umu)antj0003
(author)
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Liu, Jing-XiaUmeå universitet,Institutionen för integrativ medicinsk biologi (IMB)(Swepub:umu)liji0001
(author)
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Andersen, Peter Munch,1962-Umeå universitet,Neurovetenskaper(Swepub:umu)pean0001
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Brännström, ThomasUmeå universitet,Patologi,https://orcid.org/0000-0002-4201-8204(Swepub:umu)thbr0001
(author)
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Pedrosa Domellöf, FatimaUmeå universitet,Institutionen för integrativ medicinsk biologi (IMB),Oftalmiatrik(Swepub:umu)fape0001
(author)
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Umeå universitetInstitutionen för integrativ medicinsk biologi (IMB)
(creator_code:org_t)
Related titles
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In:Investigative Ophthalmology and Visual Science: Association for Research in Vision and Ophthalmology (ARVO)64:50146-04041552-5783
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