Reduced bioavailable manganese causes striatal urea cycle pathology in Huntington’s disease mouse model

Huntington’s disease (HD) is caused by a mutation in the huntingtin gene (HTT), resulting in profound striatal neurodegeneration through an unknown mechanism. Perturbations in the urea cycle have been reported in HD models and in HD patient blood and brain. In neurons, arginase is a central urea cyc...

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Veröffentlicht in:Biochim Biophys Acta
Hauptverfasser: Bichell, Terry Jo V., Wegrzynowicz, Michal, Tipps, K. Grace, Bradley, Emma M., Uhouse, Michael A., Bryan, Miles, Horning, Kyle, Fisher, Nicole, Dudek, Karrie, Halbesma, Timothy, Umashanker, Preethi, Stubbs, Andrew D., Holt, Hunter K., Kwakye, Gunnar F., Tidball, Andrew M., Colbran, Roger J., Aschner, Michael, Neely, M. Diana, Di Pardo, Alba, Maglione, Vittorio, Osmand, Alexander, Bowman, Aaron B.
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2017
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Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5515276/
https://ncbi.nlm.nih.gov/pubmed/28213125
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbadis.2017.02.013
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