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Related Experiment Video

Updated: May 12, 2026

Imaging Mitochondrial Ca2+ Uptake in Astrocytes and Neurons using Genetically Encoded Ca2+ Indicators (GECIs)
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Axonal excitability during ischemia in MELAS.

Karl Ng1, Kishore R Kumar, Carolyn Sue

  • 1Department of Neurology and Clinical Neurophysiology, Royal North Shore Hospital, New South Wales, Australia. kng@med.usyd.edu.au

Muscle & Nerve
|April 5, 2013
PubMed
Summary

Researchers investigated axonal excitability in patients with mitochondrial myopathy, lactic acidosis, and stroke-like episodes (MELAS) under ischemic stress. No significant differences in axonal responses were found compared to controls, suggesting distinct neuropathy mechanisms.

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Area of Science:

  • Neuroscience
  • Mitochondrial Biology
  • Neurology

Background:

  • Mitochondrial diseases like MELAS may cause energy substrate depletion, leading to ATPase-dependent pump dysfunction and membrane depolarization.
  • Axonal depolarization is observed during crises in MELAS but not typically in resting states.
  • Ischemia is used to stress axons and reveal abnormal responses, similar to observations in diabetic neuropathy.

Purpose of the Study:

  • To investigate axonal excitability in patients with MELAS under ischemic stress.
  • To determine if ischemia reveals abnormal axonal responses related to ATP-dependent mechanisms in MELAS.
  • To compare axonal responses in MELAS to those observed in diabetic neuropathy.

Main Methods:

  • Studied excitability of median nerve axons in 13 MELAS patients (6 with glucose intolerance) and 17 controls.

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  • Applied ischemia to the arm for 10 minutes by inflating a cuff.
  • Measured axonal excitability before, during, and after ischemia.
  • Main Results:

    • No significant differences were found in pre-ischemic axonal excitability between MELAS patients and controls.
    • Intra-ischemic and post-ischemic axonal responses did not differ significantly between the groups.
    • The study failed to demonstrate a defect in axonal ATP-dependent mechanisms under ischemic stress in MELAS.

    Conclusions:

    • Axonal ATP-dependent mechanisms do not appear to be detectably impaired under ischemic stress in MELAS.
    • The observed axonal depolarization during MELAS crises may involve different mechanisms than those revealed by ischemia.
    • Neuropathy mechanisms in MELAS may differ from those implicated in diabetic neuropathy.