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Protein

S-adenosylmethionine mitochondrial carrier protein

Gene

SLC25A26

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Mitochondrial solute carriers shuttle metabolites, nucleotides, and cofactors through the mitochondrial inner membrane. Specifically mediates the transport of S-adenosylmethionine (SAM) into the mitochondria.2 Publications

Enzyme regulationi

Strongly inhibited by tannic acid and Bromocresol Purple.1 Publication

GO - Molecular functioni

  • S-adenosyl-L-methionine transmembrane transporter activity Source: UniProtKB

GO - Biological processi

  • ion transport Source: Reactome
  • mitochondrial transport Source: GO_Central
  • S-adenosyl-L-methionine transport Source: UniProtKB

Keywordsi

Biological processTransport
LigandS-adenosyl-L-methionine

Enzyme and pathway databases

ReactomeiR-HSA-425393 Transport of inorganic cations/anions and amino acids/oligopeptides

Protein family/group databases

TCDBi2.A.29.18.3 the mitochondrial carrier (mc) family

Names & Taxonomyi

Protein namesi
Recommended name:
S-adenosylmethionine mitochondrial carrier protein
Alternative name(s):
Mitochondrial S-adenosylmethionine transporter
Solute carrier family 25 member 26
Gene namesi
Name:SLC25A26
Synonyms:SAMC
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000144741.17
HGNCiHGNC:20661 SLC25A26
MIMi611037 gene
neXtProtiNX_Q70HW3

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei5 – 25Helical; Name=1Sequence analysisAdd BLAST21
Transmembranei49 – 69Helical; Name=2Sequence analysisAdd BLAST21
Transmembranei85 – 105Helical; Name=3Sequence analysisAdd BLAST21
Transmembranei142 – 162Helical; Name=4Sequence analysisAdd BLAST21
Transmembranei182 – 202Helical; Name=5Sequence analysisAdd BLAST21
Transmembranei238 – 258Helical; Name=6Sequence analysisAdd BLAST21

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Combined oxidative phosphorylation deficiency 28 (COXPD28)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive mitochondrial disorder characterized by decreased activities of respiratory chain enzymes, and variable clinical manifestations. Clinical features include episodic metabolic decompensation beginning in infancy, mild muscle weakness, cardiorespiratory insufficiency, developmental delay, or even death.
See also OMIM:616794
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_076305102A → V in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025314EnsemblClinVar.1
Natural variantiVAR_076306148V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025313EnsemblClinVar.1
Natural variantiVAR_076307199P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025315EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi115286
MalaCardsiSLC25A26
MIMi616794 phenotype
OpenTargetsiENSG00000144741
PharmGKBiPA134987831

Polymorphism and mutation databases

BioMutaiSLC25A26
DMDMi74749739

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003175871 – 274S-adenosylmethionine mitochondrial carrier proteinAdd BLAST274

Proteomic databases

EPDiQ70HW3
MaxQBiQ70HW3
PaxDbiQ70HW3
PeptideAtlasiQ70HW3
PRIDEiQ70HW3
ProteomicsDBi68552
68553 [Q70HW3-2]
68554 [Q70HW3-3]

PTM databases

iPTMnetiQ70HW3
PhosphoSitePlusiQ70HW3

Expressioni

Tissue specificityi

Widely expressed. Highly expressed in testis, with moderate expression in brain, heart, kidney, lung, skeletal muscle, pancreas, small intestine and liver, and low expression in spleen.1 Publication

Gene expression databases

BgeeiENSG00000144741
CleanExiHS_SLC25A26
ExpressionAtlasiQ70HW3 baseline and differential
GenevisibleiQ70HW3 HS

Organism-specific databases

HPAiHPA026887

Interactioni

Protein-protein interaction databases

BioGridi125423, 5 interactors
STRINGi9606.ENSP00000336801

Structurei

3D structure databases

ProteinModelPortaliQ70HW3
SMRiQ70HW3
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati4 – 77Solcar 1Add BLAST74
Repeati86 – 168Solcar 2Add BLAST83
Repeati177 – 265Solcar 3Add BLAST89

Sequence similaritiesi

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0768 Eukaryota
ENOG4110BII LUCA
GeneTreeiENSGT00550000074950
HOGENOMiHOG000038810
HOVERGENiHBG108456
InParanoidiQ70HW3
KOiK15111
OMAiLKTRVML
OrthoDBiEOG091G0G6Z
PhylomeDBiQ70HW3
TreeFamiTF313186

Family and domain databases

Gene3Di1.50.40.10, 2 hits
InterProiView protein in InterPro
IPR018108 Mitochondrial_sb/sol_carrier
IPR023395 Mt_carrier_dom_sf
PfamiView protein in Pfam
PF00153 Mito_carr, 3 hits
SUPFAMiSSF103506 SSF103506, 1 hit
PROSITEiView protein in PROSITE
PS50920 SOLCAR, 3 hits

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q70HW3-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MDRPGFVAAL VAGGVAGVSV DLILFPLDTI KTRLQSPQGF SKAGGFHGIY
60 70 80 90 100
AGVPSAAIGS FPNAAAFFIT YEYVKWFLHA DSSSYLTPMK HMLAASAGEV
110 120 130 140 150
VACLIRVPSE VVKQRAQVSA STRTFQIFSN ILYEEGIQGL YRGYKSTVLR
160 170 180 190 200
EIPFSLVQFP LWESLKALWS WRQDHVVDSW QSAVCGAFAG GFAAAVTTPL
210 220 230 240 250
DVAKTRITLA KAGSSTADGN VLSVLHGVWR SQGLAGLFAG VFPRMAAISL
260 270
GGFIFLGAYD RTHSLLLEVG RKSP
Length:274
Mass (Da):29,354
Last modified:March 28, 2018 - v2
Checksum:i97315DE9CC68D19D
GO
Isoform 2 (identifier: Q70HW3-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-88: Missing.

Show »
Length:186
Mass (Da):20,116
Checksum:i8024D5D11F6CF22B
GO
Isoform 3 (identifier: Q70HW3-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-88: Missing.
     152-154: IPF → EED
     155-274: Missing.

Note: No experimental confirmation available.
Show »
Length:66
Mass (Da):7,405
Checksum:i68F03A11822C5C3E
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti208T → A in BAF84562 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_08024441S → N3 PublicationsCorresponds to variant dbSNP:rs146159281Ensembl.1
Natural variantiVAR_076305102A → V in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025314EnsemblClinVar.1
Natural variantiVAR_076306148V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025313EnsemblClinVar.1
Natural variantiVAR_076307199P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025315EnsemblClinVar.1
Natural variantiVAR_058973208T → M2 PublicationsCorresponds to variant dbSNP:rs13874Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0310621 – 88Missing in isoform 2 and isoform 3. 2 PublicationsAdd BLAST88
Alternative sequenceiVSP_031063152 – 154IPF → EED in isoform 3. 1 Publication3
Alternative sequenceiVSP_031064155 – 274Missing in isoform 3. 1 PublicationAdd BLAST120

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ580932 mRNA Translation: CAE45652.1
AK092495 mRNA Translation: BAG52559.1
AK096876 mRNA Translation: BAG53388.1
AK291873 mRNA Translation: BAF84562.1
AC145425 Genomic DNA No translation available.
AC170165 Genomic DNA No translation available.
AC092034 Genomic DNA No translation available.
AC170801 Genomic DNA No translation available.
AC235952 Genomic DNA No translation available.
AEKP01024816 Genomic DNA No translation available.
CH471055 Genomic DNA Translation: EAW65449.1
CH471055 Genomic DNA Translation: EAW65451.1
BC003399 mRNA No translation available.
BC012852 mRNA Translation: AAH12852.2
CCDSiCCDS2905.2 [Q70HW3-1]
CCDS54604.1 [Q70HW3-2]
RefSeqiNP_001158268.1, NM_001164796.1 [Q70HW3-2]
NP_775742.4, NM_173471.3 [Q70HW3-1]
XP_011531629.1, XM_011533327.2 [Q70HW3-2]
UniGeneiHs.379386

Genome annotation databases

EnsembliENST00000336733; ENSP00000336801; ENSG00000144741 [Q70HW3-2]
ENST00000354883; ENSP00000346955; ENSG00000144741 [Q70HW3-1]
ENST00000632575; ENSP00000488865; ENSG00000282739 [Q70HW3-2]
ENST00000633701; ENSP00000488659; ENSG00000282739 [Q70HW3-1]
GeneIDi115286
KEGGihsa:115286
UCSCiuc011bfq.2 human
uc011bfs.3 human [Q70HW3-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiSAMC_HUMAN
AccessioniPrimary (citable) accession number: Q70HW3
Secondary accession number(s): A8K758
, B3KRZ7, F8WAB8, Q7Z786, Q96E68
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 5, 2008
Last sequence update: March 28, 2018
Last modified: June 20, 2018
This is version 117 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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