Reactome: A Curated Pathway Database

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

Stable Identifier
R-HSA-372448
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
aspartate [mitochondrial matrix] + glutamate [cytosol] => aspartate [cytosol] + glutamate [mitochondrial matrix]
Locations in the PathwayBrowser
Summation

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
Participants
Participant Of
hasEvent
This entity is regulated by:
Title Physical Entity Activity
acidic amino acid transmembrane transporter activity of SLC25A12,13 [mitochondrial inner membrane] SLC25A12,13 [mitochondrial inner membrane] acidic amino acid transmembrane transporter activity (0015172)
Orthologous Events