Neuron-specific mitochondrial degeneration induced by hyperammonemia and octanoic acidemia

Lester R Drewes, Richard L. Leino

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20 Scopus citations


The neuropathological consequences of acute exposure to the neurotoxicants ammonia and octanoic acid were investigated with the isolated, perfused canine brain preparation. After 1 h of combined hyperammonemia and octanoic acidemia, ultrastructural changes were apparent in all brain regions examined. The cell bodies of neurons were the primary sites of these alterations. Neuronal mitochondria were distended, and the lamellae of the mitochondrial cristae were separated. In some cases the lamellae had completely dispersed, leaving only matrix remnants. Mitochondria of adjacent astrocytes appeared normal. Thus, a characteristic population of brain mitochondria is selectively vulnerable to a combination of hyperammonemia and octanoic acidemia and may be related to the biochemical mechanisms underlying encephalopathies of hepatic origin.

Original languageEnglish (US)
Pages (from-to)211-218
Number of pages8
JournalBrain Research
Issue number2
StatePublished - Aug 12 1985

Bibliographical note

Funding Information:
The authors wish to thank Audrey Comstock for preparation of the typescript and Carolyn Clark for assistance in editing and revising the manuscript. Marjorie Lindemann provided expert technical assistance. The Duluth Clinic Foundation is acknowledged for financial assistance.


  • Reye's syndrome
  • brain
  • hepatic encephalopathy
  • hyperammonemia
  • mitochondria
  • neuropathology
  • octanoic acidemia


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