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Elucidating Neuronal Susceptibility to Mitochondrial Disease

Total Cost €


EC-Contrib. €






Project "NEUROMITO" data sheet

The following table provides information about the project.


Organization address
postcode: 8290

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country Spain [ES]
 Project website
 Total cost 1˙500˙000 €
 EC max contribution 1˙500˙000 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2014-STG
 Funding Scheme ERC-STG
 Starting year 2015
 Duration (year-month-day) from 2015-05-01   to  2020-04-30


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 


 Project objective

Mitochondria generate most of the energy cells require to function. Deficits in the mitochondrial energy-generating machinery affect 1:5,000 children and cause progressive, debilitating, and usually fatal pathologies collectively known as primary mitochondrial disease. To date, there is no cure for mitochondrial disease and existing treatments are highly ineffective and mostly palliative. High-energy-requiring cells, such as neurons, are especially affected in mitochondrial disease. However, not all neuronal populations are equally affected. Furthermore, the molecular determinants of neuronal vulnerability to mitochondrial disease have not been adequately elucidated, representing a challenge for the development of efficient treatments for these pathologies. To improve on current knowledge on mitochondrial disease and to provide better therapeutic targets, this project focuses on developing ground-breaking mouse genetics and molecular biology tools that will allow the identification and dissection of the molecular determinants of neuronal vulnerability in mitochondrial disease with unprecedented definition. We will develop novel techniques to isolate both the mitochondrial and cytosolic translatome by using ribosomal tagging in vivo as well as to assess intact mitochondrial function with cell-type specificity and temporal resolution. These novel approaches will have a high impact in mitochondrial disease, with the overall aim of identifying novel therapeutic targets that will lead to effective treatments for mitochondrial disease. Furthermore, the high applicability of the tools generated will allow significant breakthroughs in the research of other pathologies with mitochondrial affectation such as diabetes or neurodegenerative processes.


year authors and title journal last update
List of publications.
2019 Elisenda Sanz, Jonathan C. Bean, Daniel P. Carey, Albert Quintana, G. Stanley McKnight
RiboTag: Ribosomal Tagging Strategy to Analyze Cell-Type-Specific mRNA Expression In Vivo
published pages: e77, ISSN: 1934-8584, DOI: 10.1002/cpns.77
Current Protocols in Neuroscience 88/1 2020-01-29
2019 Irene Bolea, Alejandro Gella, Elisenda Sanz, Patricia Prada-Dacasa, Fabien Menardy, Angela M Bard, Pablo Machuca-Márquez, Abel Eraso-Pichot, Guillem Mòdol-Caballero, Xavier Navarro, Franck Kalume, Albert Quintana
Defined neuronal populations drive fatal phenotype in a mouse model of Leigh Syndrome
published pages: , ISSN: 2050-084X, DOI: 10.7554/elife.47163
eLife 8 2020-01-29
2016 Pavel I. Zimin, Christian B. Woods, Albert Quintana, Jan-Marino Ramirez, Philip G. Morgan, Margaret M. Sedensky
Glutamatergic Neurotransmission Links Sensitivity to Volatile Anesthetics with Mitochondrial Function
published pages: 2194-2201, ISSN: 0960-9822, DOI: 10.1016/j.cub.2016.06.020
Current Biology 26/16 2020-01-29
2017 Byron Chen, Jessica Hui, Kelsey S. Montgomery, Alejandro Gella, Irene Bolea, Elisenda Sanz, Richard D. Palmiter, Albert Quintana
Loss of Mitochondrial Ndufs4 in Striatal Medium Spiny Neurons Mediates Progressive Motor Impairment in a Mouse Model of Leigh Syndrome
published pages: , ISSN: 1662-5099, DOI: 10.3389/fnmol.2017.00265
Frontiers in Molecular Neuroscience 10 2020-01-29
2017 Aundrea Rainwater, Elisenda Sanz, Richard D. Palmiter, Albert Quintana
Striatal GPR88 Modulates Foraging Efficiency
published pages: 7939-7947, ISSN: 0270-6474, DOI: 10.1523/JNEUROSCI.2439-16.2017
The Journal of Neuroscience 37/33 2020-01-29
2016 Stephanie L Padilla, Jian Qiu, Marta E Soden, Elisenda Sanz, Casey C Nestor, Forrest D Barker, Albert Quintana, Larry S Zweifel, Oline K Rønnekleiv, Martin J Kelly, Richard D Palmiter
Agouti-related peptide neural circuits mediate adaptive behaviors in the starved state
published pages: 734-741, ISSN: 1097-6256, DOI: 10.1038/nn.4274
Nature Neuroscience 19/5 2020-01-29

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