solute carrier family 25 member 1
Zuletzt aktualisiert 18. Aug 2020
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Der SLC25A1 gene provides instructions for making a protein that is found in mitochondria, which are the energy-producing centers in cells. The SLC25A1 protein transports a molecule called citrate out of mitochondria in exchange for another molecule called malate, which is transported in. Within mitochondria, both citrate and malate participate in reactions that produce energy for cell activities. Citrate is transported out of mitochondria because it also has important functions in other parts of the cell. In particular, citrate is involved in the production of fats (lipids) and the regulation of glycolysis, which is another critical energy-producing process within cells.
Normal function
Der SLC25A1 gene provides instructions for making a protein that is found in mitochondria, which are the energy-producing centers in cells. The SLC25A1 protein transports a molecule called citrate out of mitochondria in exchange for another molecule called malate, which is transported in. Within mitochondria, both citrate and malate participate in reactions that produce energy for cell activities. Citrate is transported out of mitochondria because it also has important functions in other parts of the cell. In particular, citrate is involved in the production of fats (lipids) and the regulation of glycolysis, which is another critical energy-producing process within cells.
Conditions referring to this gene or chromosome
Other names
citrate transport protein
CTP
D2L2AD
SEA
SLC20A3
solute carrier family 20 (mitochondrial citrate transporter), member 3
solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1
tricarboxylate carrier protein
tricarboxylate transport protein, mitochondrial
TXTP_HUMAN
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Weiterführende Lektüre und Referenzen
- solute carrier family 25 member 1; MedlinePlus, National Library of Medicine.
- Catalina-Rodriguez O, Kolukula VK, Tomita Y, Preet A, Palmieri F, Wellstein A, Byers S, Giaccia AJ, Glasgow E, Albanese C, Avantaggiati ML. The mitochondrial citrate transporter, CIC, is essential for mitochondrial homeostasis. Oncotarget. 2012 Oct;3(10):1220-35. doi: 10.18632/oncotarget.714.
- Iacobazzi V, Lauria G, Palmieri F. Organization and sequence of the human gene for the mitochondrial citrate transport protein. DNA Seq. 1997;7(3-4):127-39. doi: 10.3109/10425179709034029.
- Nota B, Struys EA, Pop A, Jansen EE, Fernandez Ojeda MR, Kanhai WA, Kranendijk M, van Dooren SJ, Bevova MR, Sistermans EA, Nieuwint AW, Barth M, Ben-Omran T, Hoffmann GF, de Lonlay P, McDonald MT, Meberg A, Muntau AC, Nuoffer JM, Parini R, Read MH, Renneberg A, Santer R, Strahleck T, van Schaftingen E, van der Knaap MS, Jakobs C, Salomons GS. Deficiency in SLC25A1, encoding the mitochondrial citrate carrier, causes combined D-2- and L-2-hydroxyglutaric aciduria. Am J Hum Genet. 2013 Apr 4;92(4):627-31. doi: 10.1016/j.ajhg.2013.03.009.
- Palmieri F. The mitochondrial transporter family SLC25: identification, properties and physiopathology. Mol Aspects Med. 2013 Apr-Jun;34(2-3):465-84. doi: 10.1016/j.mam.2012.05.005. Epub 2012 Dec 23.
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18. Aug 2020 | Neueste Version
1 Aug 2013 | Ursprünglich veröffentlicht
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