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Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).
Mitochondrial amino acid transporters can be subdivided on the basis of their substrates. Mitochondrial ornithine transporters play a role in the urea cycle by exchanging cytosolic ornithine (L-ornithine and D-ornithine) for mitochondrial citrulline (L-citrulline and D-citrulline) in equimolar amounts. Further members of the family include transporters of S-adenosylmethionine and carnitine.
solute carrier family 25 member 47 / SLC25A47 Show summary »« Hide summary
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solute carrier family 25 member 48 / SLC25A48 Show summary »« Hide summary
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AGC1 / SLC25A12 C Show summary »« Hide summary
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AGC2 / SLC25A13 C Show summary »« Hide summary
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GC2 (Mitochondrial glutamate carrier 2 / SLC25A18) C Show summary »« Hide summary
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GC1 (Mitochondrial glutamate carrier 1 / SLC25A22) C Show summary »« Hide summary
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ORC2 (Mitochondrial ornithine transporter 2 / SLC25A2) C Show summary »« Hide summary
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ORC1 (Mitochondrial ornithine transporter 1 / SLC25A15) C Show summary »« Hide summary
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CAC (Carnitine/acylcarnitine carrier / SLC25A20) C Show summary »« Hide summary
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ORNT3 / SLC25A29 Show summary »« Hide summary
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SLC25A38 Show summary »« Hide summary
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CGI-69 / SLC25A39 Show summary »« Hide summary
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MCFP / SLC25A40 Show summary »« Hide summary
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SLC25A44 Show summary »« Hide summary
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SLC25A45 Show summary »« Hide summary
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1. Fiermonte G, Dolce V, David L, Santorelli FM, Dionisi-Vici C, Palmieri F, Walker JE. (2003) The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms. J Biol Chem, 278 (35): 32778-83. [PMID:12807890]
2. Fiermonte G, Paradies E, Todisco S, Marobbio CM, Palmieri F. (2009) A novel member of solute carrier family 25 (SLC25A42) is a transporter of coenzyme A and adenosine 3',5'-diphosphate in human mitochondria. J Biol Chem, 284 (27): 18152-9. [PMID:19429682]
Database page citation:
Mitochondrial amino acid transporter subfamily. Accessed on 06/12/2024. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/FamilyDisplayForward?familyId=204.
Concise Guide to PHARMACOLOGY citation:
Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Transporters. Br J Pharmacol. 180 Suppl 2:S374-469.
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License
Both ornithine transporters are inhibited by the polyamine spermine [2]. Loss-of-function mutations in these genes are associated with hyperornithinemia-hyperammonemia-homocitrullinuria.