. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc Natl Acad Sci U S A. 2002 Aug 6;99(16):10671-6. PubMed.


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  1. The recent study demonstrating that tauroursodeoxycholic acid exerts significant therapeutic effects in a transgenic mouse model of Huntington's Disease (HD) is extremely intriguing. This agent was previously demonstrated to stabilize mitochondria and inhibit release of cytochrome-c, which has been linked to apoptotic cell death. This prevents activation of downstream caspases. It is possible that similar mechanisms may play a role in Alzheimer's Disease (AD) pathogenesis. There is substantial evidence showing that a number of markers for apoptotic cell death are increased in AD postmortem brain tissue. There is also substantial evidence for mitochondrial dysfunction in AD. Decreases in cytochrome oxidase activity have been demonstrated both in postmortem as well as in peripheral tissues such as platelets. Reductions in alpha-ketoglutorate dehydrogenase activity are found in both fibroblasts as well as in brain tissue.

    There is also a large body of evidence implicating increased oxidative damage in AD. All of these processes may be linked to mitochondrial dysfunction. Furthermore, mitochondrial dysfunction is linked to beta-amyloid production as recently demonstrated by Busciglio et al. Agents that can stabilize mitochondria and prevent their involvement in apoptotic cell death pathways may therefore be useful in treating a number of neurodegenerative diseases including AD.


    . Altered metabolism of the amyloid beta precursor protein is associated with mitochondrial dysfunction in Down's syndrome. Neuron. 2002 Feb 28;33(5):677-88. PubMed.

    View all comments by M. Flint Beal

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  1. Bile Acid Proves Neuroprotective in Huntington's Model