Autophagy, mitochondria and 3-nitropropionic acid joined in the same model.
Summary of "Autophagy, mitochondria and 3-nitropropionic acid joined in the same model."
Huntington's disease (HD) is a neurodegenerative disorder caused by a mutation in the gene encoding the Huntingtin protein. Although the precise mechanism by which neuronal degeneration occurs is still unclear, several elements are important to its development: 1) altered gene expression and protein synthesis, 2) mitochondrial damage and 3) the improper regulation of the autophagy programme. In this issue of British Journal of Pharmacology, Galindo and co-workers provide the first evidence for a role of the mPTP in mitochondrial fragmentation and autophagy activation. In a model of 3NP-induced cell death in human neural cells, the authors describe clear functions for mPTP and bax, but not mitochondrial fusion/fission machinery, mitochondrial fragmentation and autophagy (mitophagy). This commentary summarises the significance of this relationship and suggests several points for future development.
Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Departamento de Bioquímica y Biología Molecular y Genética, E. Enfermería y T.O., Universidad de Extremadura, CP 10003, Cáceres, Spain.
This article was published in the following journal.
Name: British journal of pharmacology
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22946678
- DOI: http://dx.doi.org/10.1111/j.1476-5381.2012.02203.x
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