Una “ambulancia” para el cerebro

Una “ambulancia” para el cerebro. IRB Barcelona Scientic News

Junto a investigadores clínicos, están preparando terapias para el glioblastoma –el cáncer cerebral más agresivo en adultos-, la Ataxia de Friedreich –una enfermedad neurodegenerativa hereditaria- y próximamente para un tipo de cáncer cerebral infantil.

An “ambulance” for the brain

An “ambulance” for the brain. IRB Barcelona Scientific News

Together with clinical researchers, they are preparing treatments for glioblastoma—the most aggressive brain cancer in adults—, Friedreich’s Ataxia—a hereditary neurodegenerative disease—, and a type of paediatric brain cancer

Applying the Retro-Enantio Approach to Obtain a Peptide Capable of Overcoming the Blood–Brain Barrier

Applying the Retro-Enantio Approach to Obtain a Peptide Capable of Overcoming the Blood–Brain Barrier. Dr. Roger Prades, Benjamí Oller-Salvia, Dr. Susanne M. Schwarzmaier, Dr. Javier Selva, Dr. María Moros, Matilde Balbi, Dr. Valeria Grazú, Dr. Jesus M. de La Fuente, Prof. Gustavo Egea, Prof. Nikolaus Plesnila, Dr. Meritxell Teixidó and Prof. Ernest Giralt; Angewandte Chemie Intl Edition (February 2015) DOI: 10.1002/anie.201411408

"In conclusion, we report a protease-resistant peptide with the capacity to transport cargos of distinct sizes and types across the BBB, one of the most restrictive barriers in the human body.
This potential BBB shuttle brings with it the possibility to fulfil an unmet clinical need, namely the treatment of CNS disorders."

Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function

Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function. Luisa Iommarini, Susana Peralta, Alessandra Torraco, Francisca Diaz, P, Mitochondrion, Available online 29 January 2015, ISSN 1567-7249, http://dx.doi.org/10.1016/j.mito.2015.01.008.


Interestingly, in contrast to what has been observed extensively in clinical studies, the mice did not show any oxidative damage. This difference could be explained because in mice the total deletion of Fxn happens in a certain tissue at a specific time, whereas FRDA is characterized by partial Frataxin deficiency in all cells throughout the lifetime.

Mitochondrial diseases Part III: Therapeutic interventions in mouse models of oxphos deficiencies

Mitochondrial diseases Part III: Therapeutic interventions in mouse models of oxphos deficiencies. Mitochondrion, Available online 29 January 2015, ISSN 1567-7249, http://dx.doi.org/10.1016/j.mito.2015.01.007.

This review summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases laying emphasis on the molecular mechanisms of action and their potential applications.

Emerging critical roles of Fe-S clusters in DNA replication and repair

Emerging critical roles of Fe-S clusters in DNA replication and repair. Jill O. Fuss, Chi-Lin Tsai, Justin P. Ishida, John A. Tainer, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 2 February 2015, ISSN 0167-4889, http://dx.doi.org/10.1016/j.bbamcr.2015.01.018.

Cellular Iron Uptake, Trafficking and Metabolism: Key Molecules and Mechanisms and their Roles in Disease

Cellular Iron Uptake, Trafficking and Metabolism: Key Molecules and Mechanisms and their Roles in Disease. D.J.R. Lane, A.M. Merlot, M.L.-H. Huang, D.-H. Bae, P.J. Jansson, S. Sahni, D.S. Kalinowski, D.R. Richardson, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 4 February 2015, ISSN 0167-4889, http://dx.doi.org/10.1016/j.bbamcr.2015.01.021.

Possible roles of frataxin in iron homeostasis under the prespective of the Friedreich's ataxia

Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency

Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency. Alain Martelli, Stéphane Schmucker, Laurence Reutenauer, Jacques R.R. Mathieu, Carole Peyssonnaux, Zoubida Karim, Hervé Puy, Bruno Galy, Matthias W. Hentze, Hélène Puccio, Cell Metabolism, Volume 21, Issue 2, 3 February 2015, Pages 311-322, ISSN 1550-4131, http://dx.doi.org/10.1016/j.cmet.2015.01.010.

The promise and perils of HDAC inhibitors in neurodegeneration

The promise and perils of HDAC inhibitors in neurodegeneration. Alessandro Didonna and
Puneet Opal; Annals of Clinical and Translational Neurology, Volume 2, Issue 1, pages 79–101, January 2015 DOI: 10.1002/acn3.147

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International Congress on Friedreich’s Ataxia and International conference on “DNA Structure in Health & Disease”

International Congress on Friedreich’s Ataxia and International conference on “DNA Structure in Health & Disease”.; 11th -13th April, 2015 at the All India Institute of Medical Sciences, New Delhi, India.