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Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease

Dmitry Lim1, Laura Fedrizzi, Marzia Tartari

  • 1Venetian Institute of Molecular Medicine, Via Orus 2, 35129 Padua, Italy.

The Journal of Biological Chemistry
|December 25, 2007
PubMed

Insights

Huntington

Area of Science:

  • Neuroscience
  • Cell Biology
  • Mitochondrial Dysfunction

Background:

  • Huntington's disease (HD) involves Ca2+ dysregulation and mitochondrial issues.
  • Striatal cells and brain tissue from HD models show altered phosphatidylinositol signaling.

Purpose of the Study:

  • Investigate Ca2+ homeostasis and mitochondrial function in Huntington's disease striatal cells.
  • Determine the impact of altered signaling pathways on cellular Ca2+ and mitochondrial health.

Main Methods:

  • Utilized immortalized striatal cells from a Huntington's disease mouse model.
  • Analyzed transcriptional changes in phosphatidylinositol cycle components and myo-inositol trisphosphate receptors.
  • Assessed mitochondrial Ca2+ handling capacity and reactive oxygen species production.

Main Results:

  • Mutant cells exhibit delayed myo-inositol trisphosphate production and reduced basal Ca2+ levels.
  • Mitochondria in mutant cells are less capable of handling high Ca2+ loads due to increased sensitivity of the permeability transition pore.
  • Increased reactive oxygen species exacerbate mitochondrial dysfunction and vulnerability.

Conclusions:

  • Altered Ca2+ homeostasis and mitochondrial dysfunction are key features in Huntington's disease striatal cells.
  • Mitochondria in HD cells are uniquely vulnerable to Ca2+ and reactive oxygen species stress.
  • Reduced cellular Ca2+ may be a compensatory mechanism to protect mitochondria from irreversible damage and cell death.

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