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• l-F i, s i, c l, c MJ, G JM, s t, M Ma, s- 
Most relevant lorenteolchahúnontrerasasareJosrauahekienaatrús
teGui J, dierssen M, Pardo b. (2013) AGC1-malate aspartate shuttle activity is critical 
scientific for dopamine handling in the nigrostriatal pathway. J. Neurochem. 124:347-62.

articles
• aMiGo i, traba J, González-barroso MM, rueda cb, Fernández M, rial e, sánchez a, satrús- 
teGui J, del arco a. (2013) Glucagon regulation of oxidative phosphorylation requires 

an increase in matrix adenine nucleotide content through Ca2+ activation of the 
mitochondrial ATP-Mg/Pi carrier SCaMC-3. J Biol Chem. 288: 7791-802.

• l-F i, r cb, a i, a a, s t, P b, s J. (2013) 
lorenteolchuedaMiGodel rcoahekiardoatrústeGui
Calcium-regulation of mitochondrial respiration maintains ATP homeostasis and re- 
quires ARALAR/AGC1-malate aspartate shuttle in intact cortical neurons. J. Neuros- 

ci. 33:13957-71.

• du J, cleGhorn W, contreras l, linton Jd, chan Gc, chertoV ao, saheki t, GoVindaraJu V, 
s M, s J, h Jb. (2013) Cytosolic reducing power preserves gluta- 
adilekatrústeGuiurley
mate in retina. Proc. Natl. Acad. Sci. U S A.110:18501-6.

•
Pla-Martín d, rueda cb, estela a, sánchez-Piris M, González-sánchez P, traba J, de la 

Fuente s, scorrano l, renau-Piqueras J, alVarez J, satrústeGui J, Palau F. (2013) Silen- 
cing of the Charcot-Marie-Tooth disease-associated gene GDAP1 induces abnormal 

mitochondrial distribution and affects Ca2+ homeostasis by reducing store-operated 
Ca2+ entry. Neurobiol Dis. 55:140-51.



ó
• The Unit has been incorporated as member of the Instituto de Investigación 
Highlights
Sanitaria Fundacin Jiménez Diaz (July 2014) and obtained a research contract 
from IIS-FJD.

•
We have advanced in the knowledge of the role of the Ca2+-dependent mito- 

chondrial metabolite carriers in mitochondrial Ca2+ signaling and in the regula- 

tion of glutamate levels in nervous tissue, and in the role of GDAP1 deficiency 
in CMT.

•
We have found that the ATP-Mg/Pi carrier SCaMC-3/Slc25a23 participates in 

the hepatic response to glucagon in vivo (Amigo et al., JBC) by transporting ad- 

enine nucleotides to the matrix, which was permissive in increasing hepatocyte 
respiratory capacity.

•
We have established the role of Ca2+ as regulator of respiration in intact neu- 

rons, independently of the effect of Ca on ATP consumption, and determined the 

relative role of SCaMC-3 and Aralar/AGC1/Slc25a12 in regulation of respiration í
and maintenance of cell ATP levels in response to different workloads (Llorente- 

Folch et al., J. Neurosc.).

•
We have developed methods to estimate stimulation of respiratory activity in 

intact cells, the use of probes (chemical or DNA-encoded) to measure mito- 
chondrial and cytosolic ATP and Ca2+ and cytosolic Na+. These methods have 13
20
been used to study physiopathological mechanisms in Charcot-Marie-Tooth dis- T 
OR
ease due to mutations in GDAP1 (in collaboration with Palau unit), revealing P
that GDAP1 deficiency results in an altered mitochondrial distribution and SOCE RE
L 
“Store-Operated Calcium Entry” activity (Pla-Martn et al., Neurobiol Dis).
A
NU
•
In collaboration with James Hurley (Univ. Washington) we have used metabo- N
 A
lomics (gas chromatography/mass spectroscopy and 13C labeling) to study the R /
role of Aralar/AGC1/Slc25a12 in intra- and intercellular traffic of amino acids E
ER
and glutamine synthesis, finding that in retina, Aralar/AGC1-malate aspartate B
CI
shuttle protects glutamate from oxidation (Du et al., PNAS).

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