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Impact of Amyotrophic Lateral Sclerosis on Slow Tonic Myofiber Composition in Human Extraocular Muscles.

Abstract 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.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title investigative ophthalmology & visual science
Publication Year Start




PMID- 28738414
OWN - NLM
STAT- MEDLINE
DA  - 20170724
DCOM- 20170728
LR  - 20170728
IS  - 1552-5783 (Electronic)
IS  - 0146-0404 (Linking)
VI  - 58
IP  - 9
DP  - 2017 Jul 01
TI  - Impact of Amyotrophic Lateral Sclerosis on Slow Tonic Myofiber Composition in
      Human Extraocular Muscles.
PG  - 3708-3715
LID - 10.1167/iovs.17-22098 [doi]
AB  - 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.
FAU - Tjust, Anton E
AU  - Tjust AE
AD  - Department of Integrative Medical Biology, Umea University, Umea, Sweden
      2Department of Clinical Science, Ophthalmology, Umea University, Umea, Sweden.
FAU - Danielsson, Adam
AU  - Danielsson A
AD  - Department of Integrative Medical Biology, Umea University, Umea, Sweden.
FAU - Andersen, Peter M
AU  - Andersen PM
AD  - Department of Pharmacology and Clinical Neuroscience, Umea University, Umea,
      Sweden.
FAU - Brannstrom, Thomas
AU  - Brannstrom T
AD  - Department of Medical Biosciences, Umea University, Umea, Sweden.
FAU - Pedrosa Domellof, Fatima
AU  - Pedrosa Domellof F
AD  - Department of Integrative Medical Biology, Umea University, Umea, Sweden
      2Department of Clinical Science, Ophthalmology, Umea University, Umea, Sweden
      3Department of Pharmacology and Clinical Neuroscience, Umea University, Umea,
      Sweden 4Department of Medical Biosciences, Umea University, Umea, Sweden.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Invest Ophthalmol Vis Sci
JT  - Investigative ophthalmology & visual science
JID - 7703701
RN  - 0 (MYH7 protein, human)
RN  - 0 (Protein Isoforms)
RN  - EC 3.6.1.- (Cardiac Myosins)
RN  - EC 3.6.4.1 (Myosin Heavy Chains)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Amyotrophic Lateral Sclerosis/*pathology
MH  - Cardiac Myosins/metabolism
MH  - Female
MH  - Fluorescent Antibody Technique, Indirect
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Muscle Fibers, Slow-Twitch/*pathology
MH  - Myofibrils/metabolism/*pathology
MH  - Myosin Heavy Chains/*metabolism
MH  - Oculomotor Muscles/metabolism/*pathology
MH  - Protein Isoforms/metabolism
EDAT- 2017/07/25 06:00
MHDA- 2017/07/29 06:00
CRDT- 2017/07/25 06:00
AID - 2645845 [pii]
AID - 10.1167/iovs.17-22098 [doi]
PST - ppublish
SO  - Invest Ophthalmol Vis Sci. 2017 Jul 1;58(9):3708-3715. doi:
      10.1167/iovs.17-22098.