April 2010
Volume 51, Issue 13
ARVO Annual Meeting Abstract  |   April 2010
Impact of Amyotrophic Lateral Sclerosis (ALS) on the Extracellular Matrix of Human Extraocular Muscles
Author Affiliations & Notes
  • J.-X. Liu
    Integrative Medical Biology, Anatomy,
    Umea University, Umea, Sweden
  • F. Pedrosa-Domellof
    Clinical Sciences/Ophthalmology,
    Umea University, Umea, Sweden
  • Footnotes
    Commercial Relationships  J.-X. Liu, None; F. Pedrosa-Domellof, None.
  • Footnotes
    Support  Swedish Research Council (63x-20399), Synfrämjandets Forskningsfond, Stiftelsen Kronprinsessan Margaretas Arbetsnämnd för Synskadade KMA, Svenska Läkarsällskapet SLS
Investigative Ophthalmology & Visual Science April 2010, Vol.51, 5826. doi:
  • Views
  • Share
  • Tools
    • Alerts
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      J.-X. Liu, F. Pedrosa-Domellof; Impact of Amyotrophic Lateral Sclerosis (ALS) on the Extracellular Matrix of Human Extraocular Muscles. Invest. Ophthalmol. Vis. Sci. 2010;51(13):5826.

      Download citation file:

      © ARVO (1962-2015); The Authors (2016-present)

  • Supplements

Purpose: : To examine the laminin isoform composition of the basement membranes (BMs) in extraocular muscle fibers, intramuscular nerves, blood vessels, capillary and neuromuscular junctions (NMJs) in normal subjects and terminal ALS patients to survey the impact of ALS in the human EOMs.

Methods: : EOM samples obtained at autopsy from 7 ALS patients and from 4 elderly controls (72-86 y) were processed for immunocytochemistry with monoclonal antibodies against laminin chains (Ln) α2, α4, α5, and β2. Neuromuscular junctions (NMJs) were identified with alpha-bungarotoxin.

Results: : The immunoreactivity with anti-Lnα2, -Lnβ2 and -Lnα5 decreased erratically in the extrasynaptic BM of EOM fibers from all patients except one whose fiber contours showed normal immunoreactivity with anti-Lnα2. Lnα4 was absent from the extrasynaptic BM of ALS EOMs. Over 1000 NMJs were examined. The majority of the NMJs did not contain Lnα2 (76.7%) or Lnβ2 (74%) but contained Lnα4 (78.9%) and Lnα5 (71.8%). The perineurium of intramuscular nerve bundles in ALS showed decreased Lnβ2. The endoneurium in ALS lacked Lnα2, α5 and β2 but had unchanged Lnα4. In most ALS muscle sections the capillaries showed normal Lnα4 and α5 but lacked Lnβ2.

Conclusions: : In terminal stages of ALS, the BM of EOM fibers, their neuromuscular junctions, nerve fibers, blood vessels and capillaries showed altered laminin chain composition, although the EOMs are remarkably spared compared to the limb muscles of these patients. These changes in laminin composition were independently regulated for laminin chain isoform and tissue location, indicating a complex impact of ALS in the human EOMs.

Keywords: extraocular muscles: structure • extracellular matrix • pathology: human 

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.