Search results for SLC25A13

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Protein (2 results from a total of 2)

Identifier: R-HSA-372485
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A13: Q9UJS0
Identifier: R-HSA-1307784
Species: Homo sapiens
Compartment: mitochondrial intermembrane space
Primary external reference: UniProt: SLC25A13: Q9UJS0

Reaction (1 results from a total of 1)

Identifier: R-HSA-372448
Species: Homo sapiens
Compartment: cytosol, mitochondrial inner membrane, mitochondrial matrix
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).
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