Premature aging changes in neuromuscular junctions of transgenic mice with an extra human CuZnSOD gene: A model for tongue pathology in Down's syndrome

Rena Yarom, Dan Sapoznikov, Yael Havivi, Karen B. Avraham, Michael Schickler, Yoram Groner

Research output: Contribution to journalArticlepeer-review

Abstract

We examined the tongue muscles in several strains of transgenic mice carrying the human Zn-Cu superoxide dismutase (CuZnSOD) gene. The presence of the extra gene was confirmed in mated progeny and the gene product activity was measured in the tongue and found to be much higher than in normal littermate controls. Using electron microscopic morphometry, the neuromuscular junctions of the transgenic mice showed significant changes resembling excessive aging, with atrophy, degeneration, withdrawal, and sometimes destruction of the terminal axons, as well as the development of multiple small terminals. The myofibers showed little change except for slight hypertrophy and an increased variability in size. They also had more megamitochondria, fat droplets and lipofuscin bodies. Excess CuZnSOD generates H2O2 and hydroxyl radicals which affect both NMJ membranes and plasticity, and which may produce premature aging. The findings resemble those observed in tongues of patients with Down's syndrome, in whom an extra CuZnSOD gene is present as part of the trisomy of chromosome 21.

Original languageEnglish
Pages (from-to)41-53
Number of pages13
JournalJournal of the Neurological Sciences
Volume88
Issue number1-3
DOIs
StatePublished - Dec 1988
Externally publishedYes

Keywords

  • CuZnSOD gene
  • NMJ morphometry
  • Premature aging
  • Transgenic mice

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