Search results for SLC25A2

Showing 14 results out of 15

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Protein (7 results from a total of 8)

Identifier: R-HSA-374020
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A2: Q9BXI2
Identifier: R-HSA-1362394
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A28: Q96A46
Identifier: R-HSA-5216091
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A22: Q9H936
Identifier: R-HSA-372474
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A21: Q9BQT8
Identifier: R-HSA-5216195
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A26: Q70HW3
Identifier: R-HSA-200557
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A20: O43772
Identifier: R-HSA-5216194
Species: Homo sapiens
Compartment: mitochondrial inner membrane
Primary external reference: UniProt: SLC25A29: Q8N8R3

Set (2 results from a total of 2)

Identifier: R-ALL-8959777
Compartment: cytosol
Identifier: R-ALL-8959775
Compartment: mitochondrial matrix

Reaction (4 results from a total of 4)

Identifier: R-HSA-8855062
Species: Homo sapiens
Compartment: cytosol, mitochondrial inner membrane, mitochondrial matrix
SLC25A26 (S-adenosylmethionine mitochondrial carrier protein) associated with the inner mitochondrial membrane mediates the exchange of cytosolic AdoMet (S-adenosylmethionine) for mitochondrial AdoHcy (S-adenosylhomocysteine). The substrate specificity and countertransport function of SLC25A26 were established from studies of liposomes reconstituted with purified protein in vitro (Agrimi et al. 2004). SLC25A26 mutations that disrupt transport activity have been identified in patients with defects of mitochondrial function, consistent with a requirement for AdoMet uptake from the cytosol to support mitochondrial methylation reactions (Kishita et al. 2015).
Identifier: R-HSA-8959781
Species: Homo sapiens
Compartment: cytosol, mitochondrial matrix, mitochondrial inner membrane
Members of the solute carrier family 25 (SLC25) can transport carboxylates, amino acids, nucleotides and cofactors across the inner mitochondrial membrane, thereby connecting cytosolic and matrix functions. The main physiological role of mitochondrial basic amino acids transporter (SLC25A29) is to carry basic amino acids into the mitochondrion. It transports arginine (L-Arg), lysine (L-Lys), homoarginine (homoArg), methylarginine (methylArg) and, to a much lesser extent, ornithine (L-Orn) and histidine (L-His) (Porcelli et al. 2014).
Identifier: R-HSA-70634
Species: Homo sapiens
Compartment: cytosol, mitochondrial inner membrane, mitochondrial matrix
The mitochondrial ornithine transporters SLC25A15 and SLC25A2 mediate the exchange of cytosolic ornithine for citrulline from the mitochondrial matrix. SLC25A15 was the first protein shown to have this function, identified because mutations in the protein are associated with elevated levels of ammonia, ornithine, and citrulline in affected individuals (Camacho et al. 1999). The second transporter, SLC25A2, identified later, is also expressed in normal cells and their apparently partly redundant function may explain the relatively mild symptoms associated with SLC25A15 deficiency compared to other defects of the urea cycle (Fiermonte et al. 2003).
Identifier: R-HSA-5652099
Species: Homo sapiens
Compartment: Golgi lumen, Golgi membrane, cytosol
The human gene SLC35A2 encodes the UDP-galactose transporter. It is located on the Golgi membrane and mediates the antiport of UDP-Gal into the Golgi lumen in exchange for UMP. Nucleotide sugars such as UDP-Gal are used in protein glycosylation in the Golgi lumen. This transporter is also known to transport UDP-N-acetylgalactosamine (UDP-GalNAc) by the same antiport mechanism. Defects in SLC35A2 limit Golgi-localised pools of UDP-Gal, resulting in truncated beta-GlcNAc-terminated N-glycans and alpha-GalNAc-terminated O-glycans. Defects in SLC35A2 can cause congenital disorder of glycosylation 2M (CDG2M; MIM:300896), a disorder characterised by developmental delay, hypotonia, ocular defects and brain malformations. Congenital disorders of glycosylation (CDGs) are generally characterised by under-glycosylated serum glycoproteins and a wide spectrum of clinical features. Mutations in SLC25A2 causing CDG2M are G8Sfs*9, V331I and M1? (Ng et al. 2013). Defects in SLC35A2 can also cause early infantile epileptic encephalopathy 22 (EIEE22; MIM:300896), a severe form of epilepsy characterised by by frequent tonic seizures or spasms beginning in infancy and accompanied by developmental problems. Mutations in SLC35A2 causing EIEE22 are Y145Pfs*76 and F324Lfs*25 (Kodera et al. 2013).

Icon (1 results from a total of 1)

Species: Homo sapiens
Curator: Bijay Jassal
Designer: Cristoffer Sevilla
SLC25A20 icon
Mitochondrial carnitine/acylcarnitine carrier protein
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