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A bile acid protects against motor and cognitive deficits and reduces striatal degeneration in the 3-nitropropionic acid model of Huntington's disease.
Exp Neurol. 2001 Oct; 171(2):351-60.EN

Abstract

There is currently no effective treatment for Huntington's disease (HD), a progressive, fatal, neurodegenerative disorder characterized by motor and cognitive deterioration. It is well established that HD is associated with perturbation of mitochondrial energy metabolism. Tauroursodeoxycholic acid (TUDCA), a naturally occurring bile acid, can stabilize the mitochondrial membrane, inhibit the mitochondrial permeability transition, decrease free radical formation, and derail apoptotic pathways. Here we report that TUDCA significantly reduced 3-nitropropionic acid (3-NP)-mediated striatal neuronal cell death in cell culture. In addition, rats treated with TUDCA exhibited an 80% reduction in apoptosis and in lesion volumes associated with 3-NP administration. Moreover, rats which received a combination of TUDCA + 3-NP exhibited sensorimotor and cognitive task performance that was indistinguishable from that of controls, and this effect persisted at least 6 months. Bile acids have traditionally been used as therapeutic agents for certain liver diseases. This is the first demonstration, however, that a bile acid can be delivered to the brain and function as a neuroprotectant and thus may offer potential therapeutic benefit in the treatment of certain neurodegenerative diseases.

Authors+Show Affiliations

Graduate Program in Neuroscience, University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

11573988

Citation

Keene, C D., et al. "A Bile Acid Protects Against Motor and Cognitive Deficits and Reduces Striatal Degeneration in the 3-nitropropionic Acid Model of Huntington's Disease." Experimental Neurology, vol. 171, no. 2, 2001, pp. 351-60.
Keene CD, Rodrigues CM, Eich T, et al. A bile acid protects against motor and cognitive deficits and reduces striatal degeneration in the 3-nitropropionic acid model of Huntington's disease. Exp Neurol. 2001;171(2):351-60.
Keene, C. D., Rodrigues, C. M., Eich, T., Linehan-Stieers, C., Abt, A., Kren, B. T., Steer, C. J., & Low, W. C. (2001). A bile acid protects against motor and cognitive deficits and reduces striatal degeneration in the 3-nitropropionic acid model of Huntington's disease. Experimental Neurology, 171(2), 351-60.
Keene CD, et al. A Bile Acid Protects Against Motor and Cognitive Deficits and Reduces Striatal Degeneration in the 3-nitropropionic Acid Model of Huntington's Disease. Exp Neurol. 2001;171(2):351-60. PubMed PMID: 11573988.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A bile acid protects against motor and cognitive deficits and reduces striatal degeneration in the 3-nitropropionic acid model of Huntington's disease. AU - Keene,C D, AU - Rodrigues,C M, AU - Eich,T, AU - Linehan-Stieers,C, AU - Abt,A, AU - Kren,B T, AU - Steer,C J, AU - Low,W C, PY - 2001/9/28/pubmed PY - 2002/1/5/medline PY - 2001/9/28/entrez SP - 351 EP - 60 JF - Experimental neurology JO - Exp Neurol VL - 171 IS - 2 N2 - There is currently no effective treatment for Huntington's disease (HD), a progressive, fatal, neurodegenerative disorder characterized by motor and cognitive deterioration. It is well established that HD is associated with perturbation of mitochondrial energy metabolism. Tauroursodeoxycholic acid (TUDCA), a naturally occurring bile acid, can stabilize the mitochondrial membrane, inhibit the mitochondrial permeability transition, decrease free radical formation, and derail apoptotic pathways. Here we report that TUDCA significantly reduced 3-nitropropionic acid (3-NP)-mediated striatal neuronal cell death in cell culture. In addition, rats treated with TUDCA exhibited an 80% reduction in apoptosis and in lesion volumes associated with 3-NP administration. Moreover, rats which received a combination of TUDCA + 3-NP exhibited sensorimotor and cognitive task performance that was indistinguishable from that of controls, and this effect persisted at least 6 months. Bile acids have traditionally been used as therapeutic agents for certain liver diseases. This is the first demonstration, however, that a bile acid can be delivered to the brain and function as a neuroprotectant and thus may offer potential therapeutic benefit in the treatment of certain neurodegenerative diseases. SN - 0014-4886 UR - https://www.unboundmedicine.com/medline/citation/11573988/A_bile_acid_protects_against_motor_and_cognitive_deficits_and_reduces_striatal_degeneration_in_the_3_nitropropionic_acid_model_of_Huntington's_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0014-4886(01)97755-6 DB - PRIME DP - Unbound Medicine ER -