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Amino Acids

, Volume 47, Issue 9, pp 1763–1777 | Cite as

Mitochondrial transporters for ornithine and related amino acids: a review

  • Magnus Monné
  • Daniela Valeria Miniero
  • Lucia Daddabbo
  • Luigi Palmieri
  • Vito Porcelli
  • Ferdinando PalmieriEmail author
Review Article
Part of the following topical collections:
  1. Homoarginine, Arginine and Relatives

Abstract

Among the members of the mitochondrial carrier family, there are transporters that catalyze the translocation of ornithine and related substrates, such as arginine, homoarginine, lysine, histidine, and citrulline, across the inner mitochondrial membrane. The mitochondrial carriers ORC1, ORC2, and SLC25A29 from Homo sapiens, BAC1 and BAC2 from Arabidopsis thaliana, and Ort1p from Saccharomyces cerevisiae have been biochemically characterized by transport assays in liposomes. All of them transport ornithine and amino acids with side chains terminating at least with one amine. There are, however, marked differences in their substrate specificities including their affinity for ornithine (KM values in the mM to μM range). These differences are most likely reflected by minor differences in the substrate binding sites of these carriers. The physiological role of the above-mentioned mitochondrial carriers is to link several metabolic pathways that take place partly in the cytosol and partly in the mitochondrial matrix and to provide basic amino acids for mitochondrial translation. In the liver, human ORC1 catalyzes the citrulline/ornithine exchange across the mitochondrial inner membrane, which is required for the urea cycle. Human ORC1, ORC2, and SLC25A29 are likely to be involved in the biosynthesis and transport of arginine, which can be used as a precursor for the synthesis of NO, agmatine, polyamines, creatine, glutamine, glutamate, and proline, as well as in the degradation of basic amino acids. BAC1 and BAC2 are implicated in some processes similar to those of their human counterparts and in nitrogen and amino acid metabolism linked to stress conditions and the development of plants. Ort1p is involved in the biosynthesis of arginine and polyamines in yeast.

Keywords

Mitochondrial carrier Mitochondrial transporter Ornithine Arginine Lysine Histidine Homoarginine Urea cycle Mitochondria Membrane transport 

Abbreviations

BAC1

Basic amino acid carrier 1

BAC2

Basic amino acid carrier 2

HHH

Hyperornithinemia-hyperammonemia-homocitrullinuria

MC

Mitochondrial carrier

NO

Nitric oxide

NOS

NO synthase

ORC1

Ornithine carrier 1

ORC2

Ornithine carrier 2

SLC25A29

Member 29 of the SLC25 protein family

Notes

Acknowledgments

This work was supported by grants from the Ministero dell’Università e della Ricerca (MIUR), the Comitato Telethon Fondazione Onlus n. GGP11139 and the Italian Human ProteomeNet no. RBRN07BMCT_009 (MIUR).

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical statement

This article is a review summarizing the results and conclusions of available publications that include previously performed studies on human or animal subjects.

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Copyright information

© Springer-Verlag Wien 2015

Authors and Affiliations

  • Magnus Monné
    • 1
    • 2
  • Daniela Valeria Miniero
    • 1
  • Lucia Daddabbo
    • 1
  • Luigi Palmieri
    • 1
  • Vito Porcelli
    • 1
  • Ferdinando Palmieri
    • 1
    Email author
  1. 1.Department of Biosciences, Biotechnologies and BiopharmaceuticsUniversity of BariBariItaly
  2. 2.Department of SciencesUniversity of BasilicataPotenzaItaly

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