TCDB is operated by the Saier Lab Bioinformatics Group
« See all members of the family


2.A.29.19.1
Mitochondrial ornithine carrier 2 (ORC2 or OrnT2) (transports ornithine, citrulline, lysine, arginine, histidine); HHH syndrome (SLC25A2). Catalyzes ornithine:citrulline antiport and ornithine:H+ antiport (Tonazzi and Indiveri, 2011).

Accession Number:Q9BXI2
Protein Name:Mitochondrial ornithine transporter 2 aka Solute carrier family 25, member 2
Length:301
Molecular Weight:32580.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:5
Location1 / Topology2 / Orientation3: Mitochondrion inner membrane1 / Multi-pass membrane protein2
Substrate Ornithine, Citrulline, lysine, Arginine, Histidine, H+

Cross database links:

Genevestigator: Q9BXI2 Q9BXI2 Q9BXI2
eggNOG: prNOG15603 NOG245037 NOG245037
HEGENOM: HBG735918 HOG000168307 HOG000168307
RefSeq: NP_114153.1   
Entrez Gene ID: 83884   
Pfam: PF00153   
OMIM: 608157  gene
KEGG: hsa:83884    hsa:83884    hsa:83884   

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:0000066 P:mitochondrial ornithine transport
GO:0055085 P:transmembrane transport
GO:0000050 P:urea cycle

References (9)

[1] “Comparative DNA sequence analysis of mouse and human protocadherin gene clusters.”  Wu Q.et.al.   11230163
[2] “Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder.”  Camacho J.A. IIIet.al.   12948741
[3] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[4] “Comparative DNA sequence analysis of mouse and human protocadherin gene clusters.”  Wu Q.et.al.   11230163
[5] “Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder.”  Camacho J.A. IIIet.al.   12948741
[6] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[7] “Comparative DNA sequence analysis of mouse and human protocadherin gene clusters.”  Wu Q.et.al.   11230163
[8] “Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder.”  Camacho J.A. IIIet.al.   12948741
[9] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334

External Searches:

  • Search: DB with
  • BLAST ExPASy (Swiss Institute of Bioinformatics (SIB) BLAST)
  • CDD Search (Conserved Domain Database)
  • Search COGs (Clusters of Orthologous Groups of proteins)
  • 2° Structure (Network Protein Sequence Analysis)

Analyze:

Predict TMSs (Predict number of transmembrane segments)
Window Size: Angle:  
Window Size: Angle:  
FASTA formatted sequence
1:	MKSGPGIQAA IDLTAGAAGG TACVLTGQPF DTIKVKMQTF PDLYKGLTDC FLKTYAQVGL 
61:	RGFYKGTGPA LMAYVAENSV LFMCYGFCQQ FVRKVAGMDK QAKLSDLQTA AAGSFASAFA 
121:	ALALCPTELV KCRLQTMYEM EMSGKIAKSH NTIWSVVKGI LKKDGPLGFY HGLSSTLLQE 
181:	VPGYFFFFGG YELSRSFFAS GRSKDELGPV HLMLSGGVAG ICLWLVVFPV DCIKSRIQVL 
241:	SMYGKQAGFI GTLLSVVRNE GIVALYSGLK ATMIRAIPAN GALFVAYEYS RKMMMKQLEA 
301:	Y