Hmdb loader
Survey
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Identification
HMDB Protein ID HMDBP02333
Secondary Accession Numbers
  • 7821
  • HMDBP07015
Name Calcium-binding mitochondrial carrier protein Aralar2
Synonyms
  1. Citrin
  2. Mitochondrial aspartate glutamate carrier 2
  3. Solute carrier family 25 member 13
Gene Name SLC25A13
Protein Type Unknown
Biological Properties
General Function Involved in transporter activity
Specific Function Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle.
Pathways Not Available
Reactions Not Available
GO Classification
Biological Process
ATP biosynthetic process
response to calcium ion
gluconeogenesis
malate-aspartate shuttle
Cellular Component
mitochondrial inner membrane
integral to plasma membrane
Component
membrane
cell part
organelle membrane
organelle inner membrane
mitochondrial inner membrane
Function
ion binding
cation binding
metal ion binding
binding
transporter activity
calcium ion binding
Molecular Function
calcium ion binding
L-aspartate transmembrane transporter activity
L-glutamate transmembrane transporter activity
Process
establishment of localization
transport
transmembrane transport
Cellular Location
  1. Mitochondrion inner membrane
  2. Multi-pass membrane protein
Gene Properties
Chromosome Location 7
Locus 7q21.3
SNPs SLC25A13
Gene Sequence
>2028 bp
ATGGCGGCCGCCAAGGTGGCTTTAACCAAGAGAGCAGATCCAGCTGAGCTTAGAACAATA
TTTTTGAAGTATGCAAGCATTGAGAAAAACGGTGAATTTTTCATGTCCCCCAATGACTTT
GTCACTCGATACTTGAACATTTTTGGAGAAAGCCAGCCTAATCCAAAGACTGTGGAACTT
TTAAGTGGAGTGGTGGATCAGACCAAAGATGGATTAATATCTTTTCAAGAATTTGTTGCC
TTTGAATCTGTCCTGTGTGCCCCTGATGCTTTGTTTATGGTAGCCTTTCAGCTGTTTGAC
AAAGCTGGCAAAGGAGAAGTAACTTTTGAGGATGTTAAGCAAGTTTTTGGACAGACCACA
ATTCATCAACATATTCCATTTAACTGGGATTCAGAATTTGTGCAACTACATTTTGGAAAA
GAAAGAAAAAGACACCTGACATATGCGGAATTTACTCAGTTTTTATTGGAAATACAACTG
GAGCACGCAAAGCAAGCCTTTGTGCAACGGGACAATGCTAGGACTGGGAGAGTCACAGCC
ATCGACTTCCGAGACATCATGGTCACCATCCGCCCCCATGTCTTGACTCCTTTTGTAGAA
GAATGTCTAGTAGCTGCTGCTGGAGGTACCACATCCCATCAAGTTAGTTTCTCCTATTTT
AATGGATTTAATTCGCTCCTTAACAACATGGAACTCATTAGAAAGATCTATAGCACTCTG
GCTGGCACCAGGAAAGATGTTGAAGTGACTAAGGAGGAGTTTGTTCTGGCAGCTCAGAAA
TTTGGTCAGGTTACACCCATGGAAGTTGACATCTTGTTTCAGTTAGCAGATTTATATGAG
CCAAGGGGACGTATGACCTTAGCAGACATTGAACGGATTGCTCCTCTGGAAGAGGGAACT
CTGCCCTTTAACTTGGCTGAGGCCCAGAGGCAGAAGGCCTCAGGTGATTCAGCTCGACCA
GTTCTTCTACAAGTTGCAGAGTCGGCCTACAGGTTTGGTCTGGGTTCTGTTGCTGGAGCT
GTTGGAGCCACTGCTGTGTATCCTATCGATCTTGTAAAAACTCGAATGCAGAACCAACGA
TCAACTGGCTCTTTTGTGGGAGAACTCATGTATAAAAACAGCTTTGACTGTTTTAAGAAA
GTGCTACGCTATGAAGGCTTCTTTGGACTGTATAGAGGTCTGTTGCCACAGTTATTGGGA
GTTGCCCCAGAGAAGGCCATAAAACTTACAGTGAACGATTTTGTGAGGGATAAATTTATG
CACAAAGATGGTTCGGTCCCACTTGCAGCAGAAATTCTTGCTGGAGGCTGCGCTGGAGGC
TCCCAGGTGATTTTCACAAATCCTTTAGAAATCGTCAAGATCCGTTTGCAAGTGGCAGGA
GAAATCACCACTGGTCCTCGAGTCAGTGCTCTGTCTGTCGTGCGGGACCTGGGGTTTTTT
GGGATCTACAAGGGTGCCAAAGCATGCTTTCTGCGGGACATTCCTTTCTCGGCCATCTAC
TTTCCGTGCTATGCTCATGTGAAGGCTTCCTTTGCAAATGAAGATGGGCAGGTTAGCCCA
GGAAGCCTGCTCTTAGCTGGTGCCATAGCTGGTATGCCTGCAGCATCTTTAGTGACCCCT
GCTGATGTTATCAAGACGAGATTACAGGTGGCTGCCCGGGCTGGCCAAACCACTTACAGC
GGAGTGATAGACTGCTTTAGAAAGATACTGCGTGAAGAAGGACCAAAAGCTCTGTGGAAG
GGAGCTGGTGCTCGTGTATTTCGATCCTCACCCCAGTTTGGTGTAACTTTGCTGACTTAC
GAATTGCTACAGCGATGGTTCTACATTGATTTTGGAGGAGTAAAACCCATGGGATCAGAG
CCAGTTCCTAAATCCAGGATCAACCTGCCTGCCCCGAATCCTGATCACGTTGGGGGCTAC
AAACTGGCAGTTGCTACATTTGCAGGGATTGAAAACAAATTTGGACTTTACCTACCTCTC
TTCAAGCCATCAGTATCTACCTCAAAGGCTATTGGTGGAGGCCCATAG
Protein Properties
Number of Residues 675
Molecular Weight 74303.085
Theoretical pI 8.616
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Calcium-binding mitochondrial carrier protein Aralar2
MAAAKVALTKRADPAELRTIFLKYASIEKNGEFFMSPNDFVTRYLNIFGESQPNPKTVEL
LSGVVDQTKDGLISFQEFVAFESVLCAPDALFMVAFQLFDKAGKGEVTFEDVKQVFGQTT
IHQHIPFNWDSEFVQLHFGKERKRHLTYAEFTQFLLEIQLEHAKQAFVQRDNARTGRVTA
IDFRDIMVTIRPHVLTPFVEECLVAAAGGTTSHQVSFSYFNGFNSLLNNMELIRKIYSTL
AGTRKDVEVTKEEFVLAAQKFGQVTPMEVDILFQLADLYEPRGRMTLADIERIAPLEEGT
LPFNLAEAQRQKASGDSARPVLLQVAESAYRFGLGSVAGAVGATAVYPIDLVKTRMQNQR
STGSFVGELMYKNSFDCFKKVLRYEGFFGLYRGLLPQLLGVAPEKAIKLTVNDFVRDKFM
HKDGSVPLAAEILAGGCAGGSQVIFTNPLEIVKIRLQVAGEITTGPRVSALSVVRDLGFF
GIYKGAKACFLRDIPFSAIYFPCYAHVKASFANEDGQVSPGSLLLAGAIAGMPAASLVTP
ADVIKTRLQVAARAGQTTYSGVIDCFRKILREEGPKALWKGAGARVFRSSPQFGVTLLTY
ELLQRWFYIDFGGVKPMGSEPVPKSRINLPAPNPDHVGGYKLAVATFAGIENKFGLYLPL
FKPSVSTSKAIGGGP
GenBank ID Protein 5052319
UniProtKB/Swiss-Prot ID Q9UJS0
UniProtKB/Swiss-Prot Entry Name CMC2_HUMAN
PDB IDs Not Available
GenBank Gene ID AF118838
GeneCard ID SLC25A13
GenAtlas ID SLC25A13
HGNC ID HGNC:10983
References
General References
  1. 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. [PubMed:15489334 ]
  2. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  3. Hillier LW, Fulton RS, Fulton LA, Graves TA, Pepin KH, Wagner-McPherson C, Layman D, Maas J, Jaeger S, Walker R, Wylie K, Sekhon M, Becker MC, O'Laughlin MD, Schaller ME, Fewell GA, Delehaunty KD, Miner TL, Nash WE, Cordes M, Du H, Sun H, Edwards J, Bradshaw-Cordum H, Ali J, Andrews S, Isak A, Vanbrunt A, Nguyen C, Du F, Lamar B, Courtney L, Kalicki J, Ozersky P, Bielicki L, Scott K, Holmes A, Harkins R, Harris A, Strong CM, Hou S, Tomlinson C, Dauphin-Kohlberg S, Kozlowicz-Reilly A, Leonard S, Rohlfing T, Rock SM, Tin-Wollam AM, Abbott A, Minx P, Maupin R, Strowmatt C, Latreille P, Miller N, Johnson D, Murray J, Woessner JP, Wendl MC, Yang SP, Schultz BR, Wallis JW, Spieth J, Bieri TA, Nelson JO, Berkowicz N, Wohldmann PE, Cook LL, Hickenbotham MT, Eldred J, Williams D, Bedell JA, Mardis ER, Clifton SW, Chissoe SL, Marra MA, Raymond C, Haugen E, Gillett W, Zhou Y, James R, Phelps K, Iadanoto S, Bubb K, Simms E, Levy R, Clendenning J, Kaul R, Kent WJ, Furey TS, Baertsch RA, Brent MR, Keibler E, Flicek P, Bork P, Suyama M, Bailey JA, Portnoy ME, Torrents D, Chinwalla AT, Gish WR, Eddy SR, McPherson JD, Olson MV, Eichler EE, Green ED, Waterston RH, Wilson RK: The DNA sequence of human chromosome 7. Nature. 2003 Jul 10;424(6945):157-64. [PubMed:12853948 ]
  4. Kobayashi K, Sinasac DS, Iijima M, Boright AP, Begum L, Lee JR, Yasuda T, Ikeda S, Hirano R, Terazono H, Crackower MA, Kondo I, Tsui LC, Scherer SW, Saheki T: The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein. Nat Genet. 1999 Jun;22(2):159-63. [PubMed:10369257 ]
  5. Del Arco A, Agudo M, Satrustegui J: Characterization of a second member of the subfamily of calcium-binding mitochondrial carriers expressed in human non-excitable tissues. Biochem J. 2000 Feb 1;345 Pt 3:725-32. [PubMed:10642534 ]
  6. Sinasac DS, Crackower MA, Lee JR, Kobayashi K, Saheki T, Scherer SW, Tsui LC: Genomic structure of the adult-onset type II citrullinemia gene, SLC25A13, and cloning and expression of its mouse homologue. Genomics. 1999 Dec 1;62(2):289-92. [PubMed:10610724 ]
  7. Palmieri L, Pardo B, Lasorsa FM, del Arco A, Kobayashi K, Iijima M, Runswick MJ, Walker JE, Saheki T, Satrustegui J, Palmieri F: Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria. EMBO J. 2001 Sep 17;20(18):5060-9. [PubMed:11566871 ]
  8. Yamaguchi N, Kobayashi K, Yasuda T, Nishi I, Iijima M, Nakagawa M, Osame M, Kondo I, Saheki T: Screening of SLC25A13 mutations in early and late onset patients with citrin deficiency and in the Japanese population: Identification of two novel mutations and establishment of multiple DNA diagnosis methods for nine mutations. Hum Mutat. 2002 Feb;19(2):122-30. [PubMed:11793471 ]