Showing Protein Mitochondrial ornithine transporter 2 (HMDBP03057)

• 8589

1. Solute carrier family 25 member 2

• 2-aminoadipic 2-oxoadipic aciduria
• Glutaric Aciduria Type I
• Hyperlysinemia I, Familial
• Hyperlysinemia II or Saccharopinuria
• Lysine degradation
• Pyridoxine dependency with seizures
• Saccharopinuria/Hyperlysinemia II

1. Mitochondrion inner membrane
2. Multi-pass membrane protein

>906 bp
ATGAAGTCCGGTCCTGGCATCCAAGCCGCCATCGACCTCACAGCGGGGGCCGCAGGGGGG
ACAGCGTGTGTACTGACTGGGCAGCCCTTCGACACAATAAAAGTGAAGATGCAGACGTTC
CCTGACCTGTACAAGGGCCTCACCGACTGCTTCCTGAAGACATACGCCCAAGTGGGTCTC
CGGGGCTTCTACAAGGGCACCGGCCCGGCACTTATGGCCTACGTCGCCGAAAACTCGGTC
CTCTTCATGTGCTACGGGTTCTGCCAGCAGTTTGTCAGGAAAGTGGCTGGAATGGACAAG
CAGGCAAAGCTGAGTGATCTCCAGACTGCAGCCGCGGGGTCCTTCGCCTCTGCATTTGCT
GCACTGGCTCTCTGCCCCACTGAGCTTGTGAAGTGCCGGCTACAGACCATGTATGAAATG
GAGATGTCAGGGAAGATAGCAAAAAGCCATAATACAATTTGGTCTGTCGTGAAGGGTATC
CTTAAAAAGGATGGCCCCTTGGGCTTCTACCATGGACTCTCGAGTACTCTACTTCAAGAA
GTACCGGGTTATTTCTTTTTCTTTGGTGGCTATGAACTGAGCCGATCGTTTTTTGCGTCA
GGGAGATCAAAAGATGAACTAGGCCCTGTCCATTTGATGTTAAGTGGTGGAGTTGCTGGA
ATTTGCCTGTGGCTTGTCGTGTTCCCAGTGGATTGTATTAAATCCAGAATTCAAGTTCTT
TCCATGTATGGGAAACAGGCAGGATTTATTGGTACCCTCTTAAGTGTTGTGAGAAATGAA
GGAATAGTAGCCTTATATTCTGGACTGAAAGCTACTATGATTCGAGCAATCCCTGCCAAT
GGGGCACTGTTTGTGGCCTACGAATACAGCAGGAAGATGATGATGAAACAGTTGGAAGCA
TACTGA

• Mito_carr (PF00153 )

>Mitochondrial ornithine transporter 2
MKSGPGIQAAIDLTAGAAGGTACVLTGQPFDTIKVKMQTFPDLYKGLTDCFLKTYAQVGL
RGFYKGTGPALMAYVAENSVLFMCYGFCQQFVRKVAGMDKQAKLSDLQTAAAGSFASAFA
ALALCPTELVKCRLQTMYEMEMSGKIAKSHNTIWSVVKGILKKDGPLGFYHGLSSTLLQE
VPGYFFFFGGYELSRSFFASGRSKDELGPVHLMLSGGVAGICLWLVVFPVDCIKSRIQVL
SMYGKQAGFIGTLLSVVRNEGIVALYSGLKATMIRAIPANGALFVAYEYSRKMMMKQLEA
Y

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3. Camacho JA, Rioseco-Camacho N, Andrade D, Porter J, Kong J: 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. Mol Genet Metab. 2003 Aug;79(4):257-71. [PubMed:12948741 ]