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2.A.29.19.2
Mitochondrial ornithine transporter (ornithine/citrulline exchanger), SLC25A15 or Orc1. Catalyzes a vital step in the urea cycle, interconnecting the cytosolic and mitochondrial components for the cycle (Moraes and Reithmeier 2012).

Accession Number:Q9Y619
Protein Name:Mitochondrial ornithine transporter 1
Length:301
Molecular Weight:32736.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:4
Location1 / Topology2 / Orientation3: Mitochondrion inner membrane1 / Multi-pass membrane protein2
Substrate Ornithine, Citrulline

Cross database links:

NOG245037NOG245037
Genevestigator: Q9Y619 Q9Y619 Q9Y619
HEGENOM: HBG735918 HOG000168307 HOG000168307
RefSeq: NP_055067.1   
Entrez Gene ID: 10166   
Pfam: PF00153   
Drugbank: Drugbank Link   
OMIM: 238970  phenotype
603861  gene
KEGG: hsa:10166    hsa:10166    hsa:10166   

Gene Ontology

GO:0016021 C:integral to membrane
GO:0005743 C:mitochondrial inner membrane
GO:0005488 F:binding
GO:0000064 F:L-ornithine transmembrane transporter activity
GO:0006520 P:cellular amino acid metabolic process
GO:0000066 P:mitochondrial ornithine transport
GO:0055085 P:transmembrane transport
GO:0000050 P:urea cycle

References (33)

[1] “Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter.”  Camacho J.A.et.al.   10369256
[2] “The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.”  Fiermonte G.et.al.   12807890
[3] “Large-scale cDNA transfection screening for genes related to cancer development and progression.”  Wan D.et.al.   15498874
[4] “The DNA sequence and analysis of human chromosome 13.”  Dunham A.et.al.   15057823
[5] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[6] “Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Tsujino S.et.al.   10805333
[7] “Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Salvi S.et.al.   11668643
[8] “Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.”  Salvi S.et.al.   11552031
[9] “A novel mutation, P126R, in a Japanese patient with HHH syndrome.”  Miyamoto T.et.al.   11814739
[10] “HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.”  Fecarotta S.et.al.   16601889
[11] “Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study.”  Tessa A.et.al.   19242930
[12] “Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter.”  Camacho J.A.et.al.   10369256
[13] “The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.”  Fiermonte G.et.al.   12807890
[14] “Large-scale cDNA transfection screening for genes related to cancer development and progression.”  Wan D.et.al.   15498874
[15] “The DNA sequence and analysis of human chromosome 13.”  Dunham A.et.al.   15057823
[16] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[17] “Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Tsujino S.et.al.   10805333
[18] “Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Salvi S.et.al.   11668643
[19] “Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.”  Salvi S.et.al.   11552031
[20] “A novel mutation, P126R, in a Japanese patient with HHH syndrome.”  Miyamoto T.et.al.   11814739
[21] “HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.”  Fecarotta S.et.al.   16601889
[22] “Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study.”  Tessa A.et.al.   19242930
[23] “Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter.”  Camacho J.A.et.al.   10369256
[24] “The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.”  Fiermonte G.et.al.   12807890
[25] “Large-scale cDNA transfection screening for genes related to cancer development and progression.”  Wan D.et.al.   15498874
[26] “The DNA sequence and analysis of human chromosome 13.”  Dunham A.et.al.   15057823
[27] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[28] “Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Tsujino S.et.al.   10805333
[29] “Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.”  Salvi S.et.al.   11668643
[30] “Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.”  Salvi S.et.al.   11552031
[31] “A novel mutation, P126R, in a Japanese patient with HHH syndrome.”  Miyamoto T.et.al.   11814739
[32] “HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.”  Fecarotta S.et.al.   16601889
[33] “Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study.”  Tessa A.et.al.   19242930

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Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MKSNPAIQAA IDLTAGAAGG TACVLTGQPF DTMKVKMQTF PDLYRGLTDC CLKTYSQVGF 
61:	RGFYKGTSPA LIANIAENSV LFMCYGFCQQ VVRKVAGLDK QAKLSDLQNA AAGSFASAFA 
121:	ALVLCPTELV KCRLQTMYEM ETSGKIAKSQ NTVWSVIKSI LRKDGPLGFY HGLSSTLLRE 
181:	VPGYFFFFGG YELSRSFFAS GRSKDELGPV PLMLSGGVGG ICLWLAVYPV DCIKSRIQVL 
241:	SMSGKQAGFI RTFINVVKNE GITALYSGLK PTMIRAFPAN GALFLAYEYS RKLMMNQLEA 
301:	Y