Mitochondrial Calcium Signalling

Activity summary

The research carried out by our group focuses on mitochondrial pathophysiology in the brain and liver, in relation to the activity of mitochondrial aspartate/glutamate transporters (AGCs). Thus, it has been established that in hepatocytes deficient in slc25a13/Citrin, the hepatic isoform causing citrullinemia type II (CTLN2), the exogenous expression of slc25a12/Aralar, the specific isoform of excitable tissues, is functional and partially reverses its phenotype. This finding opens up new therapeutic avenues for treating liver diseases caused by SLC25A13/Citrin deficiency.

Contributions to society

Citrullinemia type II (CTLN2), and neonatal intrahepatic cholestasis, caused by a deficiency in SLC25A13/Citrin, are rare diseases more frequent in different areas of Asia for which there are no effective treatments, and which require a search for new therapeutic avenues.

Collaborations

National collaborations: J.M. Cuezva (CBMSO, Autonomous University of Madrid); María José Casarejos (Ramón y Cajal Health Research Institute [IRYSCIS]); Gloria González-Aseguinolaza (CIMA; Pamplona); Alberto Paradela (CNB, CSIC); María Luz Martínez-Chantar (CiCBiogune, Bilbao).

International collaborations: E. R. S. Kunji (MRC, University of Cambridge, UK); T. Saheki (Kagoshima University, Japan).

PhD Theses

6.3.3-SEÑALIZACIÓN MITOCONDRIAL-TESIS

List of publications ordered by publication date

IF1 ablation prevents ATP synthase oligomerization, enhances mitochondrial ATP turnover and promotes an adenosine-mediated pro-inflammatory phenotype.

Domínguez-Zorita S, Romero-Carramiñana I, Santacatterina F, Esparza-Moltó PB, Simó C, Del-Arco A, Núñez de Arenas C, Saiz J, Barbas C, Cuezva JM.

Cell Death Dis. 2023 Jul 12.14(7):413.

PMID: 37433784

FI: 9

Regulation of neuronal energy metabolism by calcium: Role of MCU and Aralar/malate-aspartate shuttle.

Del Arco A, González-Moreno L, Pérez-Liébana I, Juaristi I, González-Sánchez P, Contreras L, Pardo B, Satrústegui J.

Biochim Biophys Acta Mol Cell Res. 2023 Jun.1870(5):119468.

PMID: 36997074

FI: 5,1

Exogenous aralar/slc25a12 can replace citrin/slc25a13 as malate aspartate shuttle component in liver.

González-Moreno L, Santamaría-Cano A, Paradela A, Martínez-Chantar ML, Martín MÁ, Pérez-Carreras M, García-Picazo A, Vázquez J, Calvo E, González-Aseguinolaza G, Saheki T, Del Arco A, Satrústegui J, Contreras L.

Mol Genet Metab Rep. 2023 Jun.35:100967.

PMID: 36967723

FI: 1,9

A tribute to Sebastián Cerdán and his key contributions to brain metabolism.

Satrustegui J, Larrubia PL, Rodrigues TB, Choi IY, McKenna MC.

J Neurochem. 2023 May 11.

PMID: 37169729

FI: 4,7