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Impact of Amyotroph...
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Tjust, Anton E.Umeå universitet,Institutionen för integrativ medicinsk biologi (IMB),Oftalmiatrik
(author)
Impact of Amyotrophic Lateral Sclerosis on Slow Tonic Myofiber Composition in Human Extraocular Muscles
- Article/chapterEnglish2017
Publisher, publication year, extent ...
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ASSOC RESEARCH VISION OPHTHALMOLOGY INC,2017
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electronicrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:umu-140482
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https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-140482URI
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https://doi.org/10.1167/iovs.17-22098DOI
Supplementary language notes
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Language:English
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Summary in:English
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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 analyze the proportion and cross-sectional area of myofibers containing myosin heavy chain slow-twitch (MyHCI) and myosin heavy chain slow tonic (MyHCsto) in extraocular muscles of autopsied amyotrophic lateral sclerosis (ALS) patients with either spinal or bulbar site of disease onset. METHODS. Whole-muscle cross sections from the middle portion of the medial rectus were labeled with antibodies against MyHCI or MyHCsto and laminin. Myofibers labeled with the MyHC antibodies (MyHCI+sto) and the total number of myofibers were quantified in the orbital and global layer of 6 control individuals and 18 ALS patients. The cross-sectional area of myofibers labeled for either MyHC was quantified in 130 to 472 fibers/individual in the orbital and in 180 to 573 fibers/individual in the global layer of each specimen. RESULTS. The proportion of MyHCI+sto myofibers was significantly smaller in the orbital and global layer of ALS compared to control individuals. MyHCI+sto myofibers were significantly smaller in the global layer than in the orbital layer of ALS, whereas they were of similar size in control subjects. The decreased proportion of MyHCI+sto fibers correlated significantly with the age of death, but not disease duration, in patients who had the bulbar-onset variant of ALS but not in patients with spinal variant. CONCLUSIONS. ALS, regardless of site of onset, involves a loss of myofibers containing MyHCI+sto. Only in bulbar-onset cases did aging seem to play a role in the pathophysiological processes underlying the loss of MyHCI+sto fibers.
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Danielsson, AdamUmeå universitet,Institutionen för integrativ medicinsk biologi (IMB)(Swepub:umu)adda0004
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Andersen, Peter M.Umeå universitet,Klinisk neurovetenskap(Swepub:umu)pean0001
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Brännstrom, ThomasUmeå universitet,Patologi(Swepub:umu)thbr0001
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Pedrosa-Domellöf, FatimaUmeå universitet,Oftalmiatrik,Institutionen för integrativ medicinsk biologi (IMB),Klinisk neurovetenskap,Patologi(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: ASSOC RESEARCH VISION OPHTHALMOLOGY INC58:9, s. 3708-37150146-04041552-5783
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