Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

S-adenosylmethionine mitochondrial carrier protein

Gene

SLC25A26

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

HGNCiHGNC:20661. SLC25A26.

Subcellular locationi

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

GO - Cellular componenti

  • integral component of membrane Source: GO_Central
  • mitochondrial inner membrane Source: UniProtKB
  • mitochondrion Source: HPA

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 Publication1
Natural variantiVAR_076306148V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 Publication1
Natural variantiVAR_076307199P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 Publication1

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

MaxQBiQ70HW3.
PaxDbiQ70HW3.
PeptideAtlasiQ70HW3.
PRIDEiQ70HW3.

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.
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.
PhylomeDBiQ70HW3.
TreeFamiTF313186.

Family and domain databases

Gene3Di1.50.40.10. 1 hit.
InterProiView protein in InterPro
IPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
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 NKAGGFHGIY
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):29,381
Last modified:July 5, 2004 - v1
Checksum:i21ECA1E60C9BDDAD
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_076305102A → V in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 Publication1
Natural variantiVAR_076306148V → G in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 Publication1
Natural variantiVAR_076307199P → L in COXPD28; severe abrogation of S-adenosylmethionine transport. 1 Publication1
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.
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.
XP_011531629.1. XM_011533327.2. [Q70HW3-2]
UniGeneiHs.379386.

Genome annotation databases

EnsembliENST00000336733; ENSP00000336801; ENSG00000144741. [Q70HW3-2]
ENST00000632575; ENSP00000488865; ENSG00000282739. [Q70HW3-2]
GeneIDi115286.
KEGGihsa:115286.
UCSCiuc011bfs.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, Q7Z786, Q96E68
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 5, 2008
Last sequence update: July 5, 2004
Last modified: August 30, 2017
This is version 109 of the entry and version 1 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

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families