SLC25A11
entrez
8402
canonical
uniprot
Q02978
canonical
ensembl
ENSG00000108528
resolver
ensp
ENSP00000225665
resolver
ensp
ENSP00000440804
resolver
ensp
ENSP00000458993
resolver
genesymbol
SLC25A11
resolver
genesymbol_syn
OGC
resolver
genesymbol_syn
SLC20A4
resolver
hgnc
HGNC:10981
resolver
uniprot
F5GY65
resolver
uniprot
O75537
resolver
uniprot
Q6IBH0
resolver
uniprot
Q969P7
resolver
uniprot_entry
M2OM_HUMAN
resolver
uniprot_entry
Q6IBH0_HUMAN
resolver
uniprot_sec
F5GY65
resolver
uniprot_sec
O75537
resolver
uniprot_sec
Q969P7
resolver
activity_regulation
ACTIVITY REGULATION: Proton transporter activity is activated by long-chain fatty acids/LCFAs or by chemical (2,4-dinitrophenol) protonophores. Proton transporter activity is inhibited by ATP. {ECO:0000250|UniProtKB:Q9CR62}.
uniprot
proteins
amino_acid_sequence
MAATASAGAGGIDGKPRTSPKSVKFLFGGLAGMGATVFVQPLDLVKNRMQLSGEGAKTREYKTSFHALTSILKAEGLRGIYTGLSAGLLRQATYTTTRLGIYTVLFERLTGADGTPPGFLLKAVIGMTAGATGAFVGTPAEVALIRMTADGRLPADQRRGYKNVFNALIRITREEGVLTLWRGCIPTMARAVVVNAAQLASYSQSKQFLLDSGYFSDNILCHFCASMISGLVTTAASMPVDIAKTRIQNMRMIDGKPEYKNGLDVLFKVVRYEGFFSLWKGFTPYYARLGPHTVLTFIFLEQMNKAYKRLFLSG
uniprot
proteins
disease_involvement
DISEASE: Pheochromocytoma/paraganglioma syndrome 6 (PPGL6) [MIM:618464]: A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL6 inheritance is autosomal dominant. {ECO:0000269|PubMed:29431636}. Note=Disease susceptibility is associated with variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: Catalyzes the transport of 2-oxoglutarate (alpha-oxoglutarate) across the inner mitochondrial membrane in an electroneutral exchange for malate (PubMed:25637873, PubMed:38937634). Can also exchange 2-oxoglutarate for other dicarboxylic acids such as malonate, succinate, maleate and oxaloacetate, although with lower affinity (By similarity). Substrate exchange across the membrane occurs consecutively with one substrate being transported first, then dissociating from the substrate binding site before the second substrate binds for transport in the opposite direction (PubMed:38937634). Does not transport glutathione (PubMed:25637873). In addition can facilitate proton transport in the presence of protonophores such as long-chain fatty acids, contributing to mitochondrial uncoupling and regulation of mitochondrial energetic efficiency (By similarity). Contributes to several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, gluconeogenesis from lactate, and nitrogen metabolism (By similarity). Maintains mitochondrial fusion and fission events, and the organization and morphology of cristae (PubMed:21448454). Involved in the regulation of apoptosis (By similarity). {ECO:0000250|UniProtKB:P22292, ECO:0000250|UniProtKB:P97700, ECO:0000250|UniProtKB:Q9CR62, ECO:0000269|PubMed:21448454, ECO:0000269|PubMed:25637873, ECO:0000269|PubMed:38937634}.
uniprot
proteins
mass_dalton
34062
uniprot
proteins
protein_family
Mitochondrial carrier (TC 2.A.29) family
uniprot
proteins
pubmed
1457818
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16625196
uniprot
proteins
pubmed
19413330
uniprot
proteins
pubmed
19608861
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
21448454
uniprot
proteins
pubmed
22223895
uniprot
proteins
pubmed
22814378
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25637873
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
29431636
uniprot
proteins
pubmed
37009826
uniprot
proteins
pubmed
38937634
uniprot
proteins
sequence_length
314
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250|UniProtKB:P97700}; Multi-pass membrane protein {ECO:0000255}.
uniprot
proteins
transmembrane_region
TRANSMEM 24..42; /note="Helical; Name=1"; /evidence="ECO:0000255"; TRANSMEM 83..101; /note="Helical; Name=2"; /evidence="ECO:0000255"; TRANSMEM 119..140; /note="Helical; Name=3"; /evidence="ECO:0000255"; TRANSMEM 183..202; /note="Helical; Name=4"; /evidence="ECO:0000255"; TRANSMEM 222..240; /note="Helical; Name=5"; /evidence="ECO:0000255"; TRANSMEM 281..300; /note="Helical; Name=6"; /evidence="ECO:0000255
uniprot
proteins