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SLC25:
The mitochondrial carrier family
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Human Gene
Name
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Protein
Name
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Aliases
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Predominant Substrates
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Transport type / Coupling ions *)
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Tissue distribution and cellular/subcellular
expression
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Link to disease #)
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Human gene locus
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Sequence
Accession
ID
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Splice variants and their specific features
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SLC25A1
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CIC (citrate carrier)
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CTP, tricarboxylate carrier
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citrate,
malate, PEP
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E / citrate‑H+ /
malate
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liver, kidney, pancreas (also in
brain, lung, heart) / inner mitochondrial
membrane
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22q11.21
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NM_005984
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|
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SLC25A2
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ORC2 (ornithine carrier
2)
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ORNT2
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ornithine, citrulline, lysine, arginine, histidine
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E / ornithine / citrulline + H+;
ornithine / H+
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liver, testis, spleen, lung, pancreas,
small intestine, brain, kidney / inner mitochondrial membrane
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5q31
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NM_031947
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|
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SLC25A3
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PiC (phosphate
carrier)
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PTP
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phosphate
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C / phosphate + H+
E / phosphate / OH‑
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isoform A: heart, skeletal muscle
and diaphragm; isoform B: liver, kidney, brain, thymus, lung,
heart, skeletal muscle, diaphragm / inner
mitochondrial membrane
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12q23.1
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NM_005888
isoform a
NM_002635
isoform b
NM_213612
NM_213611
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Isoforms A and B generated by alternative splicing
of exon IIIA or exon IIIB
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SLC25A4 |
AAC1 (ADP/ATP carrier 1)
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ANT 1,
T1, PEO2,
PEO3, adenine
nucleotide
translocator 1
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ADP, ATP
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E/ ADP/ATP
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heart, skeletal muscle, much less
in brain, pancreas, prostate, kidney, lung, thymus / inner mitochondrial
membrane
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AdPEOG,
Senger syndrome
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4q35.1
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NM
001151
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|
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SLC25A5
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AAC2 (ADP/ATP carrier 2)
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ANT 2,
T2
adenine
nucleotide
translocator 2
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ADP, ATP
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E/ ADP/ATP
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brain, lung, kidney, pancreas,
heart, skeletal muscle, spleen / inner mitochondrial membrane
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Xq24
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NM
001152
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| SLC25A5P1 |
|
bK250D10.4 |
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22 |
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SLC25A6
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AAC3 (ADP/ATP carrier 3)
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ANT3,
T3
adenine
nucleotide
translocator 3
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ADP, ATP
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E/ ADP/ATP
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brain, lung, kidney, liver, pancreas, heart, skeletal muscle,
spleen, thymus / inner mitochondrial membrane
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Xp22.32
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NM_001636
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| SLC25A6P1 |
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dJ1065O2.2 |
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20p11 |
NG_003107 |
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SLC25A7
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UCP1 (uncoupling
protein 1)
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thermogenin,
proton carrier
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H+
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F
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brown adipose tissue / inner mitochondrial
membrane
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4q28‑q31
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NM_021833
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|
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SLC25A8
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UCP2 (uncoupling
protein 2)
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proton carrier,
UCPH
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H+
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F
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brain, lung, kidney, spleen, heart / inner mitochondrial membrane
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obesity, type 2 diabetes
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11q13
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NM_003355
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|
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SLC25A9
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UCP3 (uncoupling
protein 3)
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proton carrier
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H+
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F
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skeletal muscle, lung / inner mitochondrial membrane
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obesity ?
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11q13
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NM_003356
NM_022803
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UCP3S, this variant (short) lacks 37 amino acids at its C
terminus
compared to UCP3L (long). UCP3S is generated when a cleavage
and
polyadenylation signal located in the last intron of the
gene prematurely terminates message elongation. Compared
to UCP3L,
UCP3S lacks the putative transmembrane domain VI and a putative
purine nucleotide-binding domain.
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SLC25A10
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DIC
(dicarboxylate carrier)
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malate, phosphate, succinate, sulphate, thiosulphate
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E / malate / phosphate
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liver, kidney, heart, brain, lung, pancreas / inner mitochondrial membrane
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17q25.3
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NM_012140
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SLC25A11
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OGC (oxoglutarate carrier)
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oxoglutarate/malate carrier
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oxoglutarate, malate
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E / oxoglutarate / malate
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heart, skeletal muscle, liver,
kidney, brain, pancreas / inner mitochondrial
membrane
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17p13.3
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NM_003562
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SLC25A12
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AGC1 (aspartate/glu‑
tamate
carrier 1)
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aralar1
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aspartate,
glutamate
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E / aspartate /
glutamate + H+
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brain, heart, skeletal muscle, lung, pancreas, kidney, but
not in liver / inner mitochondrial membrane
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2q31.1
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NM
003705
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SLC25A13
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AGC2 (aspartate/glu‑
tamate
carrier 2)
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citrin
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aspartate,
glutamate
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E / aspartate /
glutamate + H+
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liver, kidney, pancreas, heart, skeletal muscle, brain /
inner mitochondrial membrane
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Citrullinemia type II (CTLN2)G
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7q21.3
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NM
014251
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SLC25A14
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UCP5
(uncoupling
protein 5)
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BMCP1,
brain mitochondrial carrier protein
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H+ (?)
homologous to UCP1/2/3
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unknown
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widely expressed, with highest levels in brain and testis
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Xq24
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NM_003951
(long)
NM_022810
(short)
AF155810
(short + insertion)
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BCMP1S, this variant (short) lacks 3 amino acids (23-25,
VSG) compared to the transcript variant BMCP1L (long). BCMP1SI,
this variant (short + insertion) is like BCMP1S but
has a 31 amino acid insertion between putative transmembrane
segments III and IV.
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SLC25A15
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ORC1 (ornithine
carrier 1)
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ORNT1
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ornithine, citrulline, lysine, arginine
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E / ornithine / citrulline + H+;
ornithine / H+
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liver, pancreas,
lung, testis, small intestine,
spleen, kidney, brain, heart / inner mitochondrial membrane
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HHH syndrome
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13q14.11
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NM_014252
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| SLC25A15P |
pseudogene |
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Y |
NG_002817
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SLC25A16
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GDC (Graves’ disease carrier)
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GP
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CoA or a precursor of CoA (?)
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unknown
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liver, kidney, thyroid, lung, heart, skeletal muscle, brain
/ inner mitochondrial membrane
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10q21.3
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NM_152707
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SLC25A17
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ANC1 (peroxisomal adenine nucleotide carrier)
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ANT1, PMP34
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ATP
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E / ATP / AMP
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brain, kidney, lung, heart, liver, pancreas / peroxisomal
membrane
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22q13.2
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NM_006358
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SLC25A18
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GC2 (glutamate
carrier 2)
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glutamate
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C / glutamate + H+
E / glutamate / OH‑
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brain, testis, heart, pancreas, kidney, lung / inner mitochondrial
membrane
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22q11.21
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NM_031481
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SLC25A19
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DNC (deoxy‑
nucleotide carrier)
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MUP1
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dNDPs, dNTPs, NDPs, ATP,
ddNTPs
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