Mitochondrial basic amino acids transporter

Details

Name
Mitochondrial basic amino acids transporter
Synonyms
  • C14orf69
  • CACT-like
  • Carnitine/acylcarnitine translocase-like
  • Mitochondrial carnitine/acylcarnitine carrier protein CACL
  • Mitochondrial ornithine transporter 3
  • ORNT3
  • Solute carrier family 25 member 29
Gene Name
SLC25A29
Organism
Humans
Amino acid sequence
>lcl|BSEQ0000194|Mitochondrial basic amino acids transporter
MALDFLAGCAGGVAGVLVGHPFDTVKVRLQVQSVEKPQYRGTLHCFKSIIKQESVLGLYK
GLGSPLMGLTFINALVFGVQGNTLRALGHDSPLNQFLAGAAAGAIQCVICCPMELAKTRL
QLQDAGPARTYKGSLDCLAQIYGHEGLRGVNRGMVSTLLRETPSFGVYFLTYDALTRALG
CEPGDRLLVPKLLLAGGTSGIVSWLSTYPVDVVKSRLQADGLRGAPRYRGILDCVHQSYR
AEGWRVFTRGLASTLLRAFPVNAATFATVTVVLTYARGEEAGPEGEAVPAAPAGPALAQP
SSL
Number of residues
303
Molecular Weight
32061.87
Theoretical pI
8.89
GO Classification
Functions
acyl carnitine transmembrane transporter activity
Components
integral component of membrane / mitochondrial inner membrane
General Function
Acyl carnitine transmembrane transporter activity
Specific Function
Transports arginine, lysine, homoarginine, methylarginine and, to a much lesser extent, ornithine and histidine. Does not transport carnitine nor acylcarnitines. Functions by both counter-exchange and uniport mechanisms.
Pfam Domain Function
Transmembrane Regions
2-22 61-81 96-116 153-172 187-207 255-275
Cellular Location
Mitochondrion inner membrane
Gene sequence
>lcl|BSEQ0009932|Mitochondrial basic amino acids transporter (SLC25A29)
ATGGCGCTGGACTTCTTGGCTGGATGCGCGGGGGGTGTGGCAGGCGTGCTTGTGGGACAC
CCGTTTGACACGGTCAAGGTACGGCTTCAGGTCCAGAGCGTGGAGAAGCCTCAGTACCGC
GGGACGTTGCACTGCTTCAAGTCCATCATCAAGCAAGAGAGCGTGCTGGGCCTGTACAAG
GGCCTGGGCTCGCCGCTCATGGGGCTCACCTTCATCAACGCGCTGGTGTTCGGGGTGCAG
GGCAACACCCTCCGGGCCCTGGGCCACGACTCGCCCCTCAACCAGTTCCTGGCAGGTGCG
GCGGCGGGCGCCATCCAGTGCGTCATCTGCTGCCCCATGGAGCTGGCCAAGACGCGGCTG
CAGCTGCAGGACGCGGGCCCAGCGCGCACCTACAAGGGCTCGCTGGACTGCCTCGCGCAG
ATCTACGGGCACGAGGGTCTGCGTGGCGTCAACCGGGGCATGGTGTCCACGTTGCTGCGT
GAGACGCCCAGCTTCGGCGTCTACTTCCTCACCTATGACGCTCTCACGCGGGCGCTGGGC
TGCGAGCCGGGCGACCGCCTGCTGGTGCCCAAGCTGCTGTTGGCGGGCGGTACGTCAGGC
ATCGTGTCCTGGCTCTCTACCTATCCTGTGGACGTGGTCAAGTCGCGGCTGCAGGCGGAC
GGACTGCGGGGCGCCCCGCGCTACCGCGGCATCCTGGACTGCGTGCACCAGAGCTACCGC
GCCGAGGGCTGGCGCGTCTTCACACGGGGGCTGGCGTCCACGCTGCTGCGCGCCTTCCCC
GTCAACGCTGCCACCTTCGCCACCGTCACGGTGGTGCTCACCTACGCGCGCGGCGAGGAG
GCCGGGCCCGAGGGCGAGGCTGTGCCCGCCGCCCCTGCGGGGCCTGCCCTGGCGCAGCCC
TCCAGCCTGTGA
Chromosome Location
14
Locus
14q32.2
External Identifiers
ResourceLink
UniProtKB IDQ8N8R3
UniProtKB Entry NameMCATL_HUMAN
GenBank Protein ID28193150
GenBank Gene IDBX247983
GenAtlas IDSLC25A29
HGNC IDHGNC:20116
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]
  2. Heilig R, Eckenberg R, Petit JL, Fonknechten N, Da Silva C, Cattolico L, Levy M, Barbe V, de Berardinis V, Ureta-Vidal A, Pelletier E, Vico V, Anthouard V, Rowen L, Madan A, Qin S, Sun H, Du H, Pepin K, Artiguenave F, Robert C, Cruaud C, Bruls T, Jaillon O, Friedlander L, Samson G, Brottier P, Cure S, Segurens B, Aniere F, Samain S, Crespeau H, Abbasi N, Aiach N, Boscus D, Dickhoff R, Dors M, Dubois I, Friedman C, Gouyvenoux M, James R, Madan A, Mairey-Estrada B, Mangenot S, Martins N, Menard M, Oztas S, Ratcliffe A, Shaffer T, Trask B, Vacherie B, Bellemere C, Belser C, Besnard-Gonnet M, Bartol-Mavel D, Boutard M, Briez-Silla S, Combette S, Dufosse-Laurent V, Ferron C, Lechaplais C, Louesse C, Muselet D, Magdelenat G, Pateau E, Petit E, Sirvain-Trukniewicz P, Trybou A, Vega-Czarny N, Bataille E, Bluet E, Bordelais I, Dubois M, Dumont C, Guerin T, Haffray S, Hammadi R, Muanga J, Pellouin V, Robert D, Wunderle E, Gauguet G, Roy A, Sainte-Marthe L, Verdier J, Verdier-Discala C, Hillier L, Fulton L, McPherson J, Matsuda F, Wilson R, Scarpelli C, Gyapay G, Wincker P, Saurin W, Quetier F, Waterston R, Hood L, Weissenbach J: The DNA sequence and analysis of human chromosome 14. Nature. 2003 Feb 6;421(6923):601-7. Epub 2003 Jan 1. [Article]
  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  4. Camacho JA, Rioseco-Camacho N: The human and mouse SLC25A29 mitochondrial transporters rescue the deficient ornithine metabolism in fibroblasts of patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Pediatr Res. 2009 Jul;66(1):35-41. doi: 10.1203/PDR.0b013e3181a283c1. [Article]
  5. Porcelli V, Fiermonte G, Longo A, Palmieri F: The human gene SLC25A29, of solute carrier family 25, encodes a mitochondrial transporter of basic amino acids. J Biol Chem. 2014 May 9;289(19):13374-84. doi: 10.1074/jbc.M114.547448. Epub 2014 Mar 20. [Article]
  6. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00583Levocarnitineapproved, investigationalunknownDetails