Autophagy, mitochondria and 3-nitropropionic acid joined in the same model

Rosa A González-Polo, José M Bravo-San Pedro, Rubén Gómez-Sánchez, Elisa Pizarro-Estrella, Mireia Niso-Santano, José M Fuentes

OnderzoeksoutputAcademicpeer review

6 Citaten (Scopus)


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) 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 mitochondrial permeability transition pore (mPTP) in mitochondrial fragmentation and autophagy activation. In a model of cell death induced by 3-nitropropionic acid (3-NP) in human neural cells, the authors describe clear functions for mPTP and Bax, but not the mitochondrial fusion/fission machinery, mitochondrial fragmentation and autophagy (mitophagy). This commentary summarises the significance of this relationship and suggests several points for future development.

Originele taal-2English
Pagina's (van-tot)60-2
Aantal pagina's3
TijdschriftBritish Journal of Pharmacology
Nummer van het tijdschrift1
StatusPublished - jan.-2013
Extern gepubliceerdJa


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