ORT1 BASIC INFORMATION
| Standard Name | ORT1 |
|---|---|
| Systematic Name | YOR130C |
| Alias | ARG11 |
| Feature Type | ORF, Verified |
| Description | Ornithine transporter of the mitochondrial inner membrane, exports ornithine from mitochondria as part of arginine biosynthesis; human ortholog is associated with hyperammonaemia-hyperornithinaemia-homocitrullinuria (HHH) syndrome (1, 2 and see Summary Paragraph)
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| Name Description | ORnithine Transporter |
| GO Annotations | All ORT1 GO evidence and references |
|---|---|
| View Computational GO annotations for ORT1 | |
| Molecular Function | |
| Manually curated | |
| Biological Process | |
| Manually curated | |
| Cellular Component | |
| Manually curated |
| Mutant Phenotype | All ORT1 Phenotype details and references |
|---|---|
| Large-scale survey | |
| null |
| Interactions | ORT1 All interactions details and references |
|---|---|
| 5 total interaction(s) for 5 unique genes/features. | |
| Physical Interactions |
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| Genetic Interactions |
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| External Links | All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | UniProtKB |
|---|
| Primary SGDID | S000005656 |
|---|
ADDITIONAL INFORMATION for ORT1
SUMMARY PARAGRAPH for ORT1
ORT1 encodes an ornithine transporter localized to the mitochondrial inner membrane (1, 2). In yeast, ornithine is synthesized in the mitochondrial matrix and transported to the cytosol, where it is converted to arginine (3). Mutations in ORT1 cause slow growth in the absence of arginine (1); the residual growth may be due to Bac1p, another mitochondrial inner membrane transporter (4). Mutations in ORNT1, a human gene orthologous to ORT1, are associated with
REFERENCES CITED ON THIS PAGE [View Complete Literature Guide for ORT1]
| 1) | Crabeel M, et al. (1996) The ARG11 gene of Saccharomyces cerevisiae encodes a mitochondrial integral membrane protein required for arginine biosynthesis. J Biol Chem 271(40):25011-8 |
| 2) | Palmieri L, et al. (1997) Identification of the yeast ARG-11 gene as a mitochondrial ornithine carrier involved in arginine biosynthesis. FEBS Lett 410(2-3):447-51 |
| 3) | Jauniaux JC, et al. (1978) Arginine metabolism in Saccharomyces cerevisiae: subcellular localization of the enzymes. J Bacteriol 133(3):1096-1107 |
| 4) | Soetens O, et al. (1998) Transport of arginine and ornithine into isolated mitochondria of Saccharomyces cerevisiae. Eur J Biochem 258(2):702-9 |
| 5) | Camacho JA, et al. (1999) Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter. Nat Genet 22(2):151-8 |




