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Protein

Calcium-binding mitochondrial carrier protein SCaMC-1

Gene

SLC25A24

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

Functioni

Calcium-dependent mitochondrial solute carrier. Mediates the reversible, electroneutral exchange of Mg-ATP or Mg-ADP against phosphate ions, catalyzing the net uptake or efflux of adenine nucleotides across the mitochondrial inner membrane. Nucleotide transport is inactive when cytosolic calcium levels are low, and is activated by an increase in cytosolic calcium levels. May play a role in protecting cells against oxidative stress-induced cell death, probably by promoting the formation of calcium-phosphate precipitates in the mitochondrial matrix, and thereby buffering calcium levels in the mitochondrial matrix.3 Publications

Kineticsi

  1. KM=0.97 mM for AMP1 Publication
  2. KM=0.3 mM for ADP1 Publication
  3. KM=0.33 mM for ATP1 Publication
  4. KM=0.2 mM for ATP-Mg1 Publication
  5. KM=1.64 mM for Pi1 Publication
  1. Vmax=337 µmol/min/g enzyme with AMP as substrate1 Publication
  2. Vmax=345 µmol/min/g enzyme with ADP as substrate1 Publication
  3. Vmax=320 µmol/min/g enzyme with ATP as substrate1 Publication
  4. Vmax=365 µmol/min/g enzyme with ATP-Mg as substrate1 Publication
  5. Vmax=380 µmol/min/g enzyme with Pi as substrate1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Calcium bindingi32 – 431Add BLAST12
Calcium bindingi68 – 792Add BLAST12
Calcium bindingi99 – 1103Add BLAST12
Calcium bindingi135 – 1464Add BLAST12

GO - Molecular functioni

  • ATP transmembrane transporter activity Source: UniProtKB
  • calcium ion binding Source: UniProtKB

GO - Biological processi

Keywordsi

Biological processTransport
LigandCalcium, Metal-binding

Protein family/group databases

TCDBi2.A.29.23.8 the mitochondrial carrier (mc) family

Names & Taxonomyi

Protein namesi
Recommended name:
Calcium-binding mitochondrial carrier protein SCaMC-1
Alternative name(s):
Mitochondrial ATP-Mg/Pi carrier protein 1
Mitochondrial Ca(2+)-dependent solute carrier protein 1
Small calcium-binding mitochondrial carrier protein 1
Solute carrier family 25 member 24
Gene namesi
Name:SLC25A24
Synonyms:APC1, MCSC1, SCAMC1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000085491.15
HGNCiHGNC:20662 SLC25A24
MIMi608744 gene
neXtProtiNX_Q6NUK1

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
Topological domaini1 – 197Mitochondrial intermembraneSequence analysisAdd BLAST197
Transmembranei198 – 215Helical; Name=1Sequence analysisAdd BLAST18
Topological domaini216 – 252Mitochondrial matrixSequence analysisAdd BLAST37
Transmembranei253 – 272Helical; Name=2Sequence analysisAdd BLAST20
Topological domaini273 – 295Mitochondrial intermembraneSequence analysisAdd BLAST23
Transmembranei296 – 309Helical; Name=3Sequence analysisAdd BLAST14
Topological domaini310 – 345Mitochondrial matrixSequence analysisAdd BLAST36
Transmembranei346 – 365Helical; Name=4Sequence analysisAdd BLAST20
Topological domaini366 – 388Mitochondrial intermembraneSequence analysisAdd BLAST23
Transmembranei389 – 406Helical; Name=5Sequence analysisAdd BLAST18
Topological domaini407 – 445Mitochondrial matrixSequence analysisAdd BLAST39
Transmembranei446 – 465Helical; Name=6Sequence analysisAdd BLAST20
Topological domaini466 – 477Mitochondrial intermembraneSequence analysisAdd BLAST12

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Fontaine progeroid syndrome (FPS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant progeroid disorder characterized by prenatal and postnatal growth retardation, decreased subcutaneous fat tissue, wrinkled skin, an aged appearance since birth, an abnormal scalp hair pattern, sparse hair, hypoplastic distal phalanges with hypoplastic nails, a widely open anterior fontanel, facial dysmorphisms, and craniosynostosis. Early death is observed in some patients.
See also OMIM:612289
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_080617217R → C in FPS. 1 Publication1
Natural variantiVAR_080618217R → H in FPS; no effect on protein abundance; no effect on localization to the mitochondrion; altered mitochondrial ATP transport; increased sensitivity to oxidative stress that leads to mitochondrial swelling. 1 Publication1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi29957
MalaCardsiSLC25A24
MIMi612289 phenotype
OpenTargetsiENSG00000085491
Orphaneti2095 Gorlin-Chaudhry-Moss syndrome
2963 Progeroid syndrome, Petty type
PharmGKBiPA134978257

Polymorphism and mutation databases

BioMutaiSLC25A24
DMDMi167016554

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00003175941 – 477Calcium-binding mitochondrial carrier protein SCaMC-1Add BLAST477

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei320N6-acetyllysine; alternateBy similarity1
Modified residuei320N6-succinyllysine; alternateBy similarity1
Modified residuei336N6-acetyllysineCombined sources1
Modified residuei437N6-acetyllysine; alternateCombined sources1
Modified residuei437N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ6NUK1
MaxQBiQ6NUK1
PaxDbiQ6NUK1
PeptideAtlasiQ6NUK1
PRIDEiQ6NUK1
ProteomicsDBi66685
66686 [Q6NUK1-2]
TopDownProteomicsiQ6NUK1-1 [Q6NUK1-1]
Q6NUK1-2 [Q6NUK1-2]

PTM databases

iPTMnetiQ6NUK1
PhosphoSitePlusiQ6NUK1
SwissPalmiQ6NUK1

Expressioni

Tissue specificityi

Present in various cell lines (at protein level). Expressed in all tissues tested. Highly expressed in testis, expressed at intermediate level in small intestine and pancreas, and weakly expressed in kidney, spleen, liver, skeletal muscle and heart.2 Publications

Gene expression databases

BgeeiENSG00000085491 Expressed in 208 organ(s), highest expression level in colon
CleanExiHS_SLC25A24
ExpressionAtlasiQ6NUK1 baseline and differential
GenevisibleiQ6NUK1 HS

Organism-specific databases

HPAiHPA028519
HPA063636

Interactioni

Protein-protein interaction databases

BioGridi118993, 38 interactors
IntActiQ6NUK1, 7 interactors
MINTiQ6NUK1
STRINGi9606.ENSP00000457733

Structurei

Secondary structure

1477
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliQ6NUK1
SMRiQ6NUK1
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini19 – 54EF-hand 1PROSITE-ProRule annotationAdd BLAST36
Domaini55 – 88EF-hand 2PROSITE-ProRule annotationAdd BLAST34
Domaini86 – 121EF-hand 3PROSITE-ProRule annotationAdd BLAST36
Domaini122 – 157EF-hand 4PROSITE-ProRule annotationAdd BLAST36
Repeati192 – 278Solcar 1Add BLAST87
Repeati286 – 371Solcar 2Add BLAST86
Repeati383 – 471Solcar 3Add BLAST89

Domaini

The N-terminal domain can bind calcium and regulates the ATP carrier activity of the transmembrane domain. The apo form of the N-terminal domain is intrinsically disordered and binds to the transmembrane domain, leading to inhibition of the ATP carrier activity. Calcium binding leads to a major conformation change and abolishes the interaction with the transmembrane domain and the inhibition of the ATP carrier activity (PubMed:24332718).1 Publication

Sequence similaritiesi

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0036 Eukaryota
ENOG410XQ4P LUCA
GeneTreeiENSGT00880000137904
HOVERGENiHBG108464
InParanoidiQ6NUK1
KOiK14684
OMAiKVGTFER
OrthoDBiEOG091G0C8S
PhylomeDBiQ6NUK1
TreeFamiTF313492

Family and domain databases

Gene3Di1.50.40.10, 1 hit
InterProiView protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR002167 Graves_DC
IPR002067 Mit_carrier
IPR040062 Mitochondrial_carrier_protein
IPR018108 Mitochondrial_sb/sol_carrier
IPR023395 Mt_carrier_dom_sf
PANTHERiPTHR24089 PTHR24089, 1 hit
PfamiView protein in Pfam
PF13499 EF-hand_7, 2 hits
PF00153 Mito_carr, 3 hits
PRINTSiPR00928 GRAVESDC
PR00926 MITOCARRIER
SMARTiView protein in SMART
SM00054 EFh, 3 hits
SUPFAMiSSF103506 SSF103506, 1 hit
SSF47473 SSF47473, 1 hit
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 3 hits
PS50222 EF_HAND_2, 4 hits
PS50920 SOLCAR, 3 hits

Sequences (2+)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q6NUK1-1) [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
MLRWLRDFVL PTAACQDAEQ PTRYETLFQA LDRNGDGVVD IGELQEGLRN
60 70 80 90 100
LGIPLGQDAE EKIFTTGDVN KDGKLDFEEF MKYLKDHEKK MKLAFKSLDK
110 120 130 140 150
NNDGKIEASE IVQSLQTLGL TISEQQAELI LQSIDVDGTM TVDWNEWRDY
160 170 180 190 200
FLFNPVTDIE EIIRFWKHST GIDIGDSLTI PDEFTEDEKK SGQWWRQLLA
210 220 230 240 250
GGIAGAVSRT STAPLDRLKI MMQVHGSKSD KMNIFGGFRQ MVKEGGIRSL
260 270 280 290 300
WRGNGTNVIK IAPETAVKFW AYEQYKKLLT EEGQKIGTFE RFISGSMAGA
310 320 330 340 350
TAQTFIYPME VMKTRLAVGK TGQYSGIYDC AKKILKHEGL GAFYKGYVPN
360 370 380 390 400
LLGIIPYAGI DLAVYELLKS YWLDNFAKDS VNPGVMVLLG CGALSSTCGQ
410 420 430 440 450
LASYPLALVR TRMQAQAMLE GSPQLNMVGL FRRIISKEGI PGLYRGITPN
460 470
FMKVLPAVGI SYVVYENMKQ TLGVTQK
Length:477
Mass (Da):53,354
Last modified:February 5, 2008 - v2
Checksum:iF533D47B2457123F
GO
Isoform 2 (identifier: Q6NUK1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-61: MLRWLRDFVL...GIPLGQDAEE → MDSLYGDLFW...GAIQSLEEAK

Show »
Length:458
Mass (Da):51,354
Checksum:i8C70A5782DE8BF3D
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H3BMI3H3BMI3_HUMAN
Calcium-binding mitochondrial carri...
SLC25A24
69Annotation score:
J3KN42J3KN42_HUMAN
Calcium-binding mitochondrial carri...
SLC25A24
110Annotation score:

Sequence cautioni

The sequence AAF28888 differs from that shown. Reason: Frameshift at positions 418 and 464.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti75L → P in AAH68561 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_080617217R → C in FPS. 1 Publication1
Natural variantiVAR_080618217R → H in FPS; no effect on protein abundance; no effect on localization to the mitochondrion; altered mitochondrial ATP transport; increased sensitivity to oxidative stress that leads to mitochondrial swelling. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0310661 – 61MLRWL…QDAEE → MDSLYGDLFWYLDYNKDGTL DIFELQEGLEDVGAIQSLEE AK in isoform 2. 2 PublicationsAdd BLAST61

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ619987 mRNA Translation: CAF04493.1
AJ619961 mRNA Translation: CAF04058.1
AK292567 mRNA Translation: BAF85256.1
AK316304 mRNA Translation: BAH14675.1
AL359258 Genomic DNA No translation available.
AL390036 Genomic DNA No translation available.
CH471156 Genomic DNA Translation: EAW51254.1
CH471156 Genomic DNA Translation: EAW51255.1
BC014519 mRNA Translation: AAH14519.1
BC068561 mRNA Translation: AAH68561.1
AF123303 mRNA Translation: AAF28888.1 Frameshift.
CCDSiCCDS41361.1 [Q6NUK1-1]
CCDS786.1 [Q6NUK1-2]
RefSeqiNP_037518.3, NM_013386.4 [Q6NUK1-1]
NP_998816.1, NM_213651.2 [Q6NUK1-2]
UniGeneiHs.656870

Genome annotation databases

EnsembliENST00000370041; ENSP00000359058; ENSG00000085491 [Q6NUK1-2]
ENST00000565488; ENSP00000457733; ENSG00000085491 [Q6NUK1-1]
ENST00000639032; ENSP00000492810; ENSG00000284468 [Q6NUK1-2]
ENST00000640416; ENSP00000491572; ENSG00000284468 [Q6NUK1-1]
GeneIDi29957
KEGGihsa:29957
UCSCiuc001dvm.5 human [Q6NUK1-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ619987 mRNA Translation: CAF04493.1
AJ619961 mRNA Translation: CAF04058.1
AK292567 mRNA Translation: BAF85256.1
AK316304 mRNA Translation: BAH14675.1
AL359258 Genomic DNA No translation available.
AL390036 Genomic DNA No translation available.
CH471156 Genomic DNA Translation: EAW51254.1
CH471156 Genomic DNA Translation: EAW51255.1
BC014519 mRNA Translation: AAH14519.1
BC068561 mRNA Translation: AAH68561.1
AF123303 mRNA Translation: AAF28888.1 Frameshift.
CCDSiCCDS41361.1 [Q6NUK1-1]
CCDS786.1 [Q6NUK1-2]
RefSeqiNP_037518.3, NM_013386.4 [Q6NUK1-1]
NP_998816.1, NM_213651.2 [Q6NUK1-2]
UniGeneiHs.656870

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4N5XX-ray2.10A1-193[»]
4ZCUX-ray2.10A/B/C14-174[»]
4ZCVX-ray2.80A/B/C/D14-174[»]
ProteinModelPortaliQ6NUK1
SMRiQ6NUK1
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi118993, 38 interactors
IntActiQ6NUK1, 7 interactors
MINTiQ6NUK1
STRINGi9606.ENSP00000457733

Protein family/group databases

TCDBi2.A.29.23.8 the mitochondrial carrier (mc) family

PTM databases

iPTMnetiQ6NUK1
PhosphoSitePlusiQ6NUK1
SwissPalmiQ6NUK1

Polymorphism and mutation databases

BioMutaiSLC25A24
DMDMi167016554

Proteomic databases

EPDiQ6NUK1
MaxQBiQ6NUK1
PaxDbiQ6NUK1
PeptideAtlasiQ6NUK1
PRIDEiQ6NUK1
ProteomicsDBi66685
66686 [Q6NUK1-2]
TopDownProteomicsiQ6NUK1-1 [Q6NUK1-1]
Q6NUK1-2 [Q6NUK1-2]

Protocols and materials databases

DNASUi29957
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000370041; ENSP00000359058; ENSG00000085491 [Q6NUK1-2]
ENST00000565488; ENSP00000457733; ENSG00000085491 [Q6NUK1-1]
ENST00000639032; ENSP00000492810; ENSG00000284468 [Q6NUK1-2]
ENST00000640416; ENSP00000491572; ENSG00000284468 [Q6NUK1-1]
GeneIDi29957
KEGGihsa:29957
UCSCiuc001dvm.5 human [Q6NUK1-1]

Organism-specific databases

CTDi29957
DisGeNETi29957
EuPathDBiHostDB:ENSG00000085491.15
GeneCardsiSLC25A24
H-InvDBiHIX0000832
HIX0023826
HGNCiHGNC:20662 SLC25A24
HPAiHPA028519
HPA063636
MalaCardsiSLC25A24
MIMi608744 gene
612289 phenotype
neXtProtiNX_Q6NUK1
OpenTargetsiENSG00000085491
Orphaneti2095 Gorlin-Chaudhry-Moss syndrome
2963 Progeroid syndrome, Petty type
PharmGKBiPA134978257
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0036 Eukaryota
ENOG410XQ4P LUCA
GeneTreeiENSGT00880000137904
HOVERGENiHBG108464
InParanoidiQ6NUK1
KOiK14684
OMAiKVGTFER
OrthoDBiEOG091G0C8S
PhylomeDBiQ6NUK1
TreeFamiTF313492

Miscellaneous databases

ChiTaRSiSLC25A24 human
GenomeRNAii29957
PROiPR:Q6NUK1
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000085491 Expressed in 208 organ(s), highest expression level in colon
CleanExiHS_SLC25A24
ExpressionAtlasiQ6NUK1 baseline and differential
GenevisibleiQ6NUK1 HS

Family and domain databases

Gene3Di1.50.40.10, 1 hit
InterProiView protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR002167 Graves_DC
IPR002067 Mit_carrier
IPR040062 Mitochondrial_carrier_protein
IPR018108 Mitochondrial_sb/sol_carrier
IPR023395 Mt_carrier_dom_sf
PANTHERiPTHR24089 PTHR24089, 1 hit
PfamiView protein in Pfam
PF13499 EF-hand_7, 2 hits
PF00153 Mito_carr, 3 hits
PRINTSiPR00928 GRAVESDC
PR00926 MITOCARRIER
SMARTiView protein in SMART
SM00054 EFh, 3 hits
SUPFAMiSSF103506 SSF103506, 1 hit
SSF47473 SSF47473, 1 hit
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 3 hits
PS50222 EF_HAND_2, 4 hits
PS50920 SOLCAR, 3 hits
ProtoNetiSearch...

Entry informationi

Entry nameiSCMC1_HUMAN
AccessioniPrimary (citable) accession number: Q6NUK1
Secondary accession number(s): B7ZAI9
, Q5T331, Q5T485, Q6PJJ9, Q705K4, Q9P129
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 5, 2008
Last sequence update: February 5, 2008
Last modified: November 7, 2018
This is version 134 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

3D-structure, Complete proteome, Reference proteome

Documents

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