Eye muscle sparing by the muscular dystrophies: Lessons to be learned?

Francisco H. Andrade, John D. Porter, Henry J. Kaminski

Research output: Contribution to journalArticlepeer-review

53 Scopus citations

Abstract

The devastating consequences of the various muscular dystrophies are even more obvious when a muscle or muscle group is spared. The study of the exceptional cell or tissue responses may prove to be of considerable value in the analysis of disease mechanisms. The small muscles responsible for eye movements, the extraocular muscles, have functional and morphological characteristics that set them aside from other skeletal muscles. Notably, these muscles are clinically unaffected in Duchenne/Becker, limb-girdle, and congenital muscular dystrophies, pathologies due to a broken mechanical or signaling linkage between the cytoskeleton and the extracellular matrix. Uncovering the strategies used by the extraocular muscles to 'naturally' protect themselves in these diseases should contribute to knowledge of both pathogenesis and treatment. We propose that careful investigation of the cellular determinants of extraocular muscle-specific properties may provide insights into how these muscles avoid or adapt to the cascade of events leading to myofiber degeneration in the muscular dystrophies. (C) 2000 Willey-Liss, Inc.

Original languageEnglish
Pages (from-to)192-203
Number of pages12
JournalMicroscopy Research and Technique
Volume48
Issue number3-4
DOIs
StatePublished - 2000

Keywords

  • Animal models
  • Dystroglycans
  • Dystrophin
  • Extraocular muscle
  • Laminins
  • Sarcoglycans

ASJC Scopus subject areas

  • Anatomy
  • Histology
  • Instrumentation
  • Medical Laboratory Technology

Fingerprint

Dive into the research topics of 'Eye muscle sparing by the muscular dystrophies: Lessons to be learned?'. Together they form a unique fingerprint.

Cite this