Page 125 - CIBERER2016-ENG

Most relevant scientific articles
• Nagamori S., Wiriyasermkul P., Guarch M.E., Okuyama H., Nakagomi S., Tadagaki K. et al. Novel cystine transporter in renal proximal tubule identified as a missing partner of cystinuria-related plasma membrane protein rBAT/SLC3A1. Proceedings of the National Academy of Sciences of the United States of America. 2016;113(3):775-780.
• De La Ballina L.R., Cano-Crespo S., Gonzalez-Munoz E., Bial S., Estrach S., Cailleteau L. et al. Amino acid transport associated to cluster of differentiation 98 heavy chain (CD98HC) is at the cross-road of oxidative stress and amino acid availability. Journal of Biological Chemistry. 2016;291(18):9700-9711.
• Rodriguez-Banqueri A., Errasti-Murugarren E., Bartoccioni P., Kowalczyk L., Peralvarez-Marin A., Palacin M. et al. Stabilization of a prokaryotic LAT transporter by random mutagenesis. Journal of General Physiology. 2016;147(4):353-368.
• Sebastian D., Sorianello E., Segales J., Irazoki A., Ruiz-Bonilla V., Sala D. et al. Mfn2 deficiency links age-related sarcopenia and impaired autophagy to activation of an adaptive mitophagy pathway. EMBO Journal. 2016.
• Cormerais Y., Giuliano S., LeFloch R., Front B., Durivault J., Tambutte E. et al. Genetic disruption of the multifunctional CD98/LAT1 complex demonstrates the key role of essential amino acid transport in the control of mTORC1 and tumor growth. Cancer Research. 2016;76(15):4481-4492.
Hightlights
Our activity has been focused in four research lines. At first instance, in collaboration with Virginia Nunes (U730) we have identified the second amino acid transporter associated with the heavy subunit rBAT (AGT1) (Nagamori et al., PNAS 2016). Because AGT1 transports cysteine and is expressed in the proximal straight tubule is a candidate transporter to have a role in cystinuria.
Secondly, we have demonstrated a key role of CD98hc, the heavy subunit of amino acid transporters involved in inherited rare diseases like lysinuric protein intolerance (y+LAT1/CD98hc) and autism (LAT1/ CD98hc), in oxidative stress, essential amino acid availability and harmonization of the amino acid content (basic and neutral) in the cell (De la Ballina et al., JBC; Cormerais et al., Cancer Res, 2016).
In third place, we have stablished a strategy based on random mutagenesis to improve the stability of membrane proteins for structural biology studies (crystallography and X ray diffraction) (Rodríguez- Banqueri et al. General Physiology 2016).
Finally, we have demonstrated that ablation of Mfn2, related to the rare inherited disease Charcot-Marie- Tooth, inhibits autophagy and causes age-related sarcopenia (Sebastian et al., PNAS 2016).
At present, we run joint projects in collaboration with Virginia Nunes U730, Isabel Varela U761 and Rafael Artuch U703 in the study of the role of amino acid transporters in hearing loss, cataracts and neurological disorders.
ER
research groups 125