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UniProtKB - Q70HW3 (SAMC_HUMAN)
Protein
S-adenosylmethionine mitochondrial carrier protein
Gene
SLC25A26
Organism
Homo sapiens (Human)
Status
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 PublicationsActivity 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
- S-adenosyl-L-methionine transport Source: UniProtKB
Keywordsi
Biological process | Transport |
Ligand | S-adenosyl-L-methionine |
Enzyme and pathway databases
PathwayCommonsi | Q70HW3 |
Reactomei | R-HSA-425393, Transport of inorganic cations/anions and amino acids/oligopeptides |
SignaLinki | Q70HW3 |
Protein family/group databases
TCDBi | 2.A.29.18.3, the mitochondrial carrier (mc) family |
Names & Taxonomyi
Protein namesi | Recommended name: S-adenosylmethionine mitochondrial carrier proteinAlternative name(s): Mitochondrial S-adenosylmethionine transporter Solute carrier family 25 member 26 |
Gene namesi | Name:SLC25A26 Synonyms:SAMC |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:20661, SLC25A26 |
MIMi | 611037, gene |
neXtProti | NX_Q70HW3 |
VEuPathDBi | HostDB:ENSG00000144741 |
Subcellular locationi
Mitochondrion
- Mitochondrion inner membrane 1 Publication; Multi-pass membrane protein 1 Publication
Mitochondrion
- mitochondrial inner membrane Source: UniProtKB
- mitochondrion Source: HPA
Other locations
- integral component of membrane Source: UniProtKB-KW
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transmembranei | 5 – 25 | Helical; Name=1Sequence analysisAdd BLAST | 21 | |
Transmembranei | 49 – 69 | Helical; Name=2Sequence analysisAdd BLAST | 21 | |
Transmembranei | 85 – 105 | Helical; Name=3Sequence analysisAdd BLAST | 21 | |
Transmembranei | 142 – 162 | Helical; Name=4Sequence analysisAdd BLAST | 21 | |
Transmembranei | 182 – 202 | Helical; Name=5Sequence analysisAdd BLAST | 21 | |
Transmembranei | 238 – 258 | Helical; Name=6Sequence analysisAdd BLAST | 21 |
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion inner membranePathology & Biotechi
Involvement in diseasei
Combined oxidative phosphorylation deficiency 28 (COXPD28)1 Publication
The disease is caused by variants 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.
Related information in OMIMFeature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_076305 | 102 | A → V in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025314EnsemblClinVar. | 1 | |
Natural variantiVAR_076306 | 148 | V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025313EnsemblClinVar. | 1 | |
Natural variantiVAR_076307 | 199 | P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025315EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variant, Primary mitochondrial diseaseOrganism-specific databases
DisGeNETi | 115286 |
MalaCardsi | SLC25A26 |
MIMi | 616794, phenotype |
OpenTargetsi | ENSG00000144741 |
Orphaneti | 466784, Neonatal severe cardiopulmonary failure due to mitochondrial methylation defect |
PharmGKBi | PA134987831 |
Miscellaneous databases
Pharosi | Q70HW3, Tbio |
Genetic variation databases
BioMutai | SLC25A26 |
DMDMi | 74749739 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000317587 | 1 – 274 | S-adenosylmethionine mitochondrial carrier proteinAdd BLAST | 274 |
Proteomic databases
EPDi | Q70HW3 |
jPOSTi | Q70HW3 |
MassIVEi | Q70HW3 |
MaxQBi | Q70HW3 |
PaxDbi | Q70HW3 |
PeptideAtlasi | Q70HW3 |
PRIDEi | Q70HW3 |
ProteomicsDBi | 30470 68552 [Q70HW3-1] 68553 [Q70HW3-2] 68554 [Q70HW3-3] |
PTM databases
iPTMneti | Q70HW3 |
PhosphoSitePlusi | Q70HW3 |
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
Bgeei | ENSG00000144741, Expressed in epithelium of mammary gland and 207 other tissues |
ExpressionAtlasi | Q70HW3, baseline and differential |
Genevisiblei | Q70HW3, HS |
Organism-specific databases
HPAi | ENSG00000144741, Low tissue specificity |
Interactioni
Protein-protein interaction databases
BioGRIDi | 125423, 6 interactors |
IntActi | Q70HW3, 1 interactor |
STRINGi | 9606.ENSP00000346955 |
Miscellaneous databases
RNActi | Q70HW3, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Repeati | 4 – 77 | Solcar 1Add BLAST | 74 | |
Repeati | 86 – 168 | Solcar 2Add BLAST | 83 | |
Repeati | 177 – 265 | Solcar 3Add BLAST | 89 |
Sequence similaritiesi
Keywords - Domaini
Repeat, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG0768, Eukaryota |
GeneTreei | ENSGT00550000074950 |
InParanoidi | Q70HW3 |
OMAi | PRVGWIS |
PhylomeDBi | Q70HW3 |
TreeFami | TF313186 |
Family and domain databases
Gene3Di | 1.50.40.10, 1 hit |
InterProi | View protein in InterPro IPR018108, Mitochondrial_sb/sol_carrier IPR023395, Mt_carrier_dom_sf |
Pfami | View protein in Pfam PF00153, Mito_carr, 3 hits |
SUPFAMi | SSF103506, SSF103506, 1 hit |
PROSITEi | View protein in PROSITE PS50920, SOLCAR, 3 hits |
s (3+)i Sequence
Sequence statusi: Complete.
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 10 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: Q70HW3-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
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
Computationally mapped potential isoform sequencesi
There are 10 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketH7C430 | H7C430_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 210 | Annotation score: | ||
H0YCZ5 | H0YCZ5_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 86 | Annotation score: | ||
H0YF50 | H0YF50_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 91 | Annotation score: | ||
A0A8I5KPW2 | A0A8I5KPW2_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 73 | Annotation score: | ||
A0A8I5KQI7 | A0A8I5KQI7_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 93 | Annotation score: | ||
A0A8I5KRE2 | A0A8I5KRE2_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 88 | Annotation score: | ||
A0A8I5KS24 | A0A8I5KS24_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 171 | Annotation score: | ||
A0A8I5KTP7 | A0A8I5KTP7_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 135 | Annotation score: | ||
A0A8I5KW47 | A0A8I5KW47_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 154 | Annotation score: | ||
A0A8I5QKW7 | A0A8I5QKW7_HUMAN | S-adenosylmethionine mitochondrial ... | SLC25A26 | 185 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 208 | T → A in BAF84562 (PubMed:14702039).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_080244 | 41 | S → N3 PublicationsCorresponds to variant dbSNP:rs146159281EnsemblClinVar. | 1 | |
Natural variantiVAR_076305 | 102 | A → V in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025314EnsemblClinVar. | 1 | |
Natural variantiVAR_076306 | 148 | V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025313EnsemblClinVar. | 1 | |
Natural variantiVAR_076307 | 199 | P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 PublicationCorresponds to variant dbSNP:rs869025315EnsemblClinVar. | 1 | |
Natural variantiVAR_058973 | 208 | T → M2 PublicationsCorresponds to variant dbSNP:rs13874EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_031062 | 1 – 88 | Missing in isoform 2 and isoform 3. 2 PublicationsAdd BLAST | 88 | |
Alternative sequenceiVSP_031063 | 152 – 154 | IPF → EED in isoform 3. 1 Publication | 3 | |
Alternative sequenceiVSP_031064 | 155 – 274 | Missing in isoform 3. 1 PublicationAdd BLAST | 120 |
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 |
CCDSi | CCDS2905.2 [Q70HW3-1] CCDS54604.1 [Q70HW3-2] |
RefSeqi | NP_001158268.1, NM_001164796.1 [Q70HW3-2] NP_775742.4, NM_173471.3 [Q70HW3-1] XP_011531629.1, XM_011533327.2 [Q70HW3-2] |
Genome annotation databases
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
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 |
CCDSi | CCDS2905.2 [Q70HW3-1] CCDS54604.1 [Q70HW3-2] |
RefSeqi | NP_001158268.1, NM_001164796.1 [Q70HW3-2] NP_775742.4, NM_173471.3 [Q70HW3-1] XP_011531629.1, XM_011533327.2 [Q70HW3-2] |
3D structure databases
AlphaFoldDBi | Q70HW3 |
SMRi | Q70HW3 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 125423, 6 interactors |
IntActi | Q70HW3, 1 interactor |
STRINGi | 9606.ENSP00000346955 |
Protein family/group databases
TCDBi | 2.A.29.18.3, the mitochondrial carrier (mc) family |
PTM databases
iPTMneti | Q70HW3 |
PhosphoSitePlusi | Q70HW3 |
Genetic variation databases
BioMutai | SLC25A26 |
DMDMi | 74749739 |
Proteomic databases
EPDi | Q70HW3 |
jPOSTi | Q70HW3 |
MassIVEi | Q70HW3 |
MaxQBi | Q70HW3 |
PaxDbi | Q70HW3 |
PeptideAtlasi | Q70HW3 |
PRIDEi | Q70HW3 |
ProteomicsDBi | 30470 68552 [Q70HW3-1] 68553 [Q70HW3-2] 68554 [Q70HW3-3] |
Protocols and materials databases
Antibodypediai | 15419, 109 antibodies from 18 providers |
DNASUi | 115286 |
Genome annotation databases
Organism-specific databases
CTDi | 115286 |
DisGeNETi | 115286 |
GeneCardsi | SLC25A26 |
HGNCi | HGNC:20661, SLC25A26 |
HPAi | ENSG00000144741, Low tissue specificity |
MalaCardsi | SLC25A26 |
MIMi | 611037, gene 616794, phenotype |
neXtProti | NX_Q70HW3 |
OpenTargetsi | ENSG00000144741 |
Orphaneti | 466784, Neonatal severe cardiopulmonary failure due to mitochondrial methylation defect |
PharmGKBi | PA134987831 |
VEuPathDBi | HostDB:ENSG00000144741 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0768, Eukaryota |
GeneTreei | ENSGT00550000074950 |
InParanoidi | Q70HW3 |
OMAi | PRVGWIS |
PhylomeDBi | Q70HW3 |
TreeFami | TF313186 |
Enzyme and pathway databases
PathwayCommonsi | Q70HW3 |
Reactomei | R-HSA-425393, Transport of inorganic cations/anions and amino acids/oligopeptides |
SignaLinki | Q70HW3 |
Miscellaneous databases
BioGRID-ORCSi | 115286, 344 hits in 1096 CRISPR screens |
ChiTaRSi | SLC25A26, human |
GenomeRNAii | 115286 |
Pharosi | Q70HW3, Tbio |
PROi | PR:Q70HW3 |
RNActi | Q70HW3, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000144741, Expressed in epithelium of mammary gland and 207 other tissues |
ExpressionAtlasi | Q70HW3, baseline and differential |
Genevisiblei | Q70HW3, HS |
Family and domain databases
Gene3Di | 1.50.40.10, 1 hit |
InterProi | View protein in InterPro IPR018108, Mitochondrial_sb/sol_carrier IPR023395, Mt_carrier_dom_sf |
Pfami | View protein in Pfam PF00153, Mito_carr, 3 hits |
SUPFAMi | SSF103506, SSF103506, 1 hit |
PROSITEi | View protein in PROSITE PS50920, SOLCAR, 3 hits |
MobiDBi | Search... |
Entry informationi
Entry namei | SAMC_HUMAN | |
Accessioni | Q70HW3Primary (citable) accession number: Q70HW3 Secondary accession number(s): A8K758 Q96E68 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 5, 2008 |
Last sequence update: | March 28, 2018 | |
Last modified: | May 25, 2022 | |
This is version 139 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Reference proteomeDocuments
- Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families