Energy dysfunction in Huntington’s disease: insights from PGC-1α, AMPK, and CKB
| العنوان: | Energy dysfunction in Huntington’s disease: insights from PGC-1α, AMPK, and CKB |
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| المؤلفون: | Yow-Sien Lin, Yijuang Chern, Tz-Chuen Ju |
| المصدر: | Cellular and Molecular Life Sciences. 69:4107-4120 |
| بيانات النشر: | Springer Science and Business Media LLC, 2012. |
| سنة النشر: | 2012 |
| مصطلحات موضوعية: | medicine.medical_specialty, Huntingtin, AMP-Activated Protein Kinases, Biology, Models, Biological, Energy homeostasis, Mice, Cellular and Molecular Neuroscience, Atrophy, Huntington's disease, Internal medicine, Creatine Kinase, BB Form, mental disorders, medicine, Animals, Humans, Dementia, Cognitive decline, Protein kinase A, Molecular Biology, Heat-Shock Proteins, Pharmacology, Cell Biology, Creatine, medicine.disease, Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha, Huntington Disease, Endocrinology, Molecular Medicine, Signal transduction, Energy Metabolism, Signal Transduction, Transcription Factors |
| الوصف: | Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a CAG trinucleotide expansion in the Huntingtin (Htt) gene. When the number of CAG repeats exceeds 36, the translated polyglutamine-expanded Htt protein interferes with the normal functions of many types of cellular machinery and causes cytotoxicity. Clinical symptoms include progressive involuntary movement disorders, psychiatric signs, cognitive decline, dementia, and a shortened lifespan. The most severe brain atrophy is observed in the striatum and cortex. Besides the well-characterized neuronal defects, recent studies showed that the functions of mitochondria and several key players in energy homeostasis are abnormally regulated during HD progression. Energy dysregulation thus is now recognized as an important pathogenic pathway of HD. This review focuses on the importance of three key molecular determinants (peroxisome proliferator-activated receptor-γ coactivator-1α, AMP-activated protein kinase, and creatine kinase B) of cellular energy homeostasis and their possible involvement in HD pathogenesis. |
| تدمد: | 1420-9071 1420-682X |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0f42365f55375fab3398decd8aed9363Test https://doi.org/10.1007/s00018-012-1025-2Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....0f42365f55375fab3398decd8aed9363 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14209071 1420682X |
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