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

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

Stable Identifier

R-HSA-372448

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial inner membrane, mitochondrial matrix

Synonyms

aspartate [mitochondrial matrix] + glutamate [cytosol] => aspartate [cytosol] + glutamate [mitochondrial matrix]

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

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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
10642534	Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues del Arco, A, Agudo, M, Satrústegui, J	Biochem J	2000
11566871	Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria Palmieri, L, Pardo, B, Lasorsa, FM, del Arco, A, Kobayashi, K, Iijima, M, Runswick, MJ, Walker, JE, Saheki, T, Satrústegui, J, Palmieri, F	EMBO J	2001
12602510	Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency Saheki, T, Kobayashi, K, Iijima, M, Nishi, I, Yasuda, T, Yamaguchi, N, Gao, HZ, Jalil, MA, Begum, L, Li, MX	Metab Brain Dis	2002
10369257	The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein Kobayashi, K, Sinasac, DS, Iijima, M, Boright, AP, Begum, L, Lee, JR, Yasuda, T, Ikeda, S, Hirano, R, Terazono, H, Crackower, MA, Kondo, I, Tsui, LC, Scherer, SW, Saheki, T	Nat Genet	1999