SLC25A12,13 exchange cytosolic L-Glu for mitochondrial matrix L-Asp

Stable Identifier
Reaction [transition]
Homo sapiens
aspartate [mitochondrial matrix] + glutamate [cytosol] => aspartate [cytosol] + glutamate [mitochondrial matrix]
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Calcium-binding mitochondrial carrier proteins Aralar1 and Aralar2 (SLC25A12 and SLC25A13 respectively), located in the inner mitochondrial membrane, mediate the exchange of cytosolic aspartate and mitochondrial glutamate (Palmieri et al. 2001). The exchange is physiologically irreversible because of the potential across the inner mitochondrial membrane (positive outside, negative inside). In the body, SLC25A12 is found mainly in heart, skeletal muscle, and brain, while SCL25A13 is widely expressed but most abundant in liver (del Arco et al. 2000; Palmieri et al. 2001). Defects in SLC25A13 are associated with type II citrullinemia, characterised by a liver-specific deficiency of the urea cycle enzyme argininosuccinate synthase (Kobayashi et al. 1999, Saheki et al. 2002).

Literature References
PubMed ID Title Journal Year
11566871 Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria

del Arco, A, Kobayashi, K, Saheki, T, Iijima, M, Walker, JE, Runswick, MJ, Lasorsa, FM, Palmieri, L, Palmieri, F, Pardo, B, Satrústegui, J

EMBO J 2001
10642534 Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues

del Arco, A, Satrústegui, J, Agudo, M

Biochem J 2000
12602510 Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency

Kobayashi, K, Saheki, T, Begum, L, Iijima, M, Jalil, MA, Li, MX, Yasuda, T, Gao, HZ, Yamaguchi, N, Nishi, I

Metab Brain Dis 2002
10369257 The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein

Kobayashi, K, Scherer, SW, Kondo, I, Ikeda, S, Crackower, MA, Iijima, M, Boright, AP, Hirano, R, Lee, JR, Tsui, LC, Saheki, T, Begum, L, Sinasac, DS, Terazono, H, Yasuda, T

Nat Genet 1999
Catalyst Activity

acidic amino acid transmembrane transporter activity of SLC25A12,13 [mitochondrial inner membrane]

Orthologous Events
Cross References
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