Our research focuses on Friedreich's ataxia, a hereditary disorder impacting muscle coordination. We aim to comprehend its molecular origins and develop novel therapies. This condition results from frataxin deficiency, a crucial mitochondrial protein. In addition to neurodegeneration, it can lead to heart issues and diabetes. We utilize cellular models to investigate the impact of frataxin loss on neurons and glial cells, testing therapeutic approaches to address functional deficits.

Using Conditional transgenic models for the study of neurodegenerative diseases

Ca2+ entry in mitochondria through the Ca2+ uniporter (CaU) is important in cell Ca2+ signaling, but its persistence in mitochondria is associated with mitochondrial dysfunction and cell death. We are interested in the study of systems for Ca2+ signaling in mitochondria that do not require Ca2+ entry in the organelle, the mitochondrial carriers of aspartate-glutamate carriers (AGC) aralar and citrin, and of ATP-Mg/Pi, or Short CaMCs (SCaMCs).

We use the extreme thermophilic bacteria Thermus thermophilus (Tth) as our main laboratory model. Unlike any other extreme thermophile, Tth grows fast at 70 ºC on LB-like medium, forms colonies on plates, and shows a very efficient natural competence system. The greater ability to crystallize of its enzymes compared to their mesophilic counterparts and its ancient phylogenetic origin gives high biological and evolutionary interest to this model.

Streptomyces and yeasts produce a high number of molecules with biotechnological value. We study the expression molecular mechanism of proteins implicated in some of this biosynthetic process, and try to obtain new molecules with possible industrial applicability. During the last years, we have been studying the biosynthetic cluster of the nucleoside antibiotic A201A (ata) from Streptomyces capreolus, and some yeast proteins with glycosyltransferase activity able to produce prebiotic oligosaccharides.

The research activity of our group has been focused on molecular aspects of the protist Leishmania and the immune-pathology that the infection of this parasite causes. On the one hand, we are studying molecular processes associated with the peculiar mechanisms of gene expression in an organism in which transcriptional regulation is almost absent.

The group belongs to the Biomedical Network Research Centre for Rare Diseases (CIBERER) and to Hospital La Paz Institute for Health Research (IdiPaz). Our work is focused on rare neurometabolic diseases, performing translational studies by the generation and charactetrization of cellular and animal models of disease, as research tools to understand underlying molecular and physiopathological mechanismsm, to identify biomarkers and novel therapeutical targets.

The research work is mainly aimed at understanding the structure-activity relations of biological macromolecules, as well as at studying the transient protein-protein and protein-nucleic acid interactions – which are crucial in many cell processes. We also try to get a deeper insight into the post-translational modifications of proteins and, in particular, of mitochondrial cytochrome c (Cc) – a protein playing a double role in redox metabolism and programmed cell death (PCD) – upon chemical modification by nitration and phosphorylation.

The regulatory mechanisms orchestrated by non-coding RNAs have emerged in recent years as ubiquitous in all living beings. In particular, bacterial responses to virtually any stress involve alterations in the abundance of certain species of small RNAs and/or antisense RNAs (noncoding strand). Our group aims to identify and analyze the regulatory mechanisms mediated by non-coding RNAs in cyanobacteria.