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Protein

Creatine kinase S-type, mitochondrial

Gene

CKMT2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Reversibly catalyzes the transfer of phosphate between ATP and various phosphogens (e.g. creatine phosphate). Creatine kinase isoenzymes play a central role in energy transduction in tissues with large, fluctuating energy demands, such as skeletal muscle, heart, brain and spermatozoa.

Catalytic activityi

ATP + creatine = ADP + phosphocreatine.PROSITE-ProRule annotation

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei225 – 2251ATP
Binding sitei270 – 2701ATPPROSITE-ProRule annotation
Binding sitei326 – 3261ATP
Binding sitei369 – 3691ATP

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi162 – 1665ATP
Nucleotide bindingi354 – 3596ATP

GO - Molecular functioni

  1. ATP binding Source: UniProtKB-KW
  2. creatine kinase activity Source: ProtInc

GO - Biological processi

  1. cellular nitrogen compound metabolic process Source: Reactome
  2. creatine metabolic process Source: Reactome
  3. muscle contraction Source: ProtInc
  4. small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Kinase, Transferase

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS05555-MONOMER.
ReactomeiREACT_813. Creatine metabolism.

Names & Taxonomyi

Protein namesi
Recommended name:
Creatine kinase S-type, mitochondrial (EC:2.7.3.2)
Alternative name(s):
Basic-type mitochondrial creatine kinase
Short name:
Mib-CK
Sarcomeric mitochondrial creatine kinase
Short name:
S-MtCK
Gene namesi
Name:CKMT2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 5

Organism-specific databases

HGNCiHGNC:1996. CKMT2.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Reactome
  2. mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA26534.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3939Mitochondrion1 PublicationAdd
BLAST
Chaini40 – 419380Creatine kinase S-type, mitochondrialPRO_0000016594Add
BLAST

Proteomic databases

MaxQBiP17540.
PaxDbiP17540.
PRIDEiP17540.

2D gel databases

UCD-2DPAGEP17540.

PTM databases

PhosphoSiteiP17540.

Expressioni

Tissue specificityi

Sarcomere-specific. Found only in heart and skeletal muscles.

Gene expression databases

BgeeiP17540.
CleanExiHS_CKMT2.
ExpressionAtlasiP17540. baseline and differential.
GenevestigatoriP17540.

Organism-specific databases

HPAiHPA051880.

Interactioni

Subunit structurei

Exists as an octamer composed of four CKMT2 homodimers.1 Publication

Protein-protein interaction databases

BioGridi107580. 8 interactions.
IntActiP17540. 6 interactions.
MINTiMINT-5004086.
STRINGi9606.ENSP00000254035.

Structurei

Secondary structure

1
419
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi50 – 534Combined sources
Helixi63 – 675Combined sources
Helixi70 – 767Combined sources
Helixi87 – 9610Combined sources
Helixi115 – 1184Combined sources
Helixi120 – 13011Combined sources
Turni131 – 1333Combined sources
Turni136 – 1383Combined sources
Beta strandi165 – 1695Combined sources
Turni177 – 1793Combined sources
Helixi182 – 19716Combined sources
Helixi201 – 2033Combined sources
Beta strandi205 – 2106Combined sources
Helixi215 – 2239Combined sources
Helixi234 – 2374Combined sources
Turni238 – 24811Combined sources
Beta strandi250 – 2545Combined sources
Beta strandi257 – 27317Combined sources
Helixi280 – 30021Combined sources
Turni309 – 3113Combined sources
Helixi318 – 3203Combined sources
Beta strandi326 – 3327Combined sources
Helixi334 – 3385Combined sources
Helixi342 – 3487Combined sources
Beta strandi351 – 3555Combined sources
Beta strandi367 – 3737Combined sources
Beta strandi376 – 3783Combined sources
Helixi380 – 3889Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2GL6X-ray2.30A/B/C/D/E/F/G/H27-416[»]
ProteinModelPortaliP17540.
SMRiP17540. Positions 47-413.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP17540.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini46 – 13287Phosphagen kinase N-terminalPROSITE-ProRule annotationAdd
BLAST
Domaini159 – 401243Phosphagen kinase C-terminalPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni40 – 6425Cardiolipin-bindingBy similarityAdd
BLAST

Sequence similaritiesi

Belongs to the ATP:guanido phosphotransferase family.PROSITE-ProRule annotation
Contains 1 phosphagen kinase C-terminal domain.PROSITE-ProRule annotation
Contains 1 phosphagen kinase N-terminal domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG3869.
GeneTreeiENSGT00550000074561.
HOGENOMiHOG000232165.
HOVERGENiHBG001339.
InParanoidiP17540.
KOiK00933.
OMAiMTSIESK.
OrthoDBiEOG7XM2XW.
PhylomeDBiP17540.
TreeFamiTF314214.

Family and domain databases

Gene3Di1.10.135.10. 1 hit.
3.30.590.10. 1 hit.
InterProiIPR000749. ATP-guanido_PTrfase.
IPR022415. ATP-guanido_PTrfase_AS.
IPR022414. ATP-guanido_PTrfase_cat.
IPR022413. ATP-guanido_PTrfase_N.
IPR014746. Gln_synth/guanido_kin_cat_dom.
[Graphical view]
PANTHERiPTHR11547. PTHR11547. 1 hit.
PfamiPF00217. ATP-gua_Ptrans. 1 hit.
PF02807. ATP-gua_PtransN. 1 hit.
[Graphical view]
SUPFAMiSSF48034. SSF48034. 1 hit.
PROSITEiPS00112. PHOSPHAGEN_KINASE. 1 hit.
PS51510. PHOSPHAGEN_KINASE_C. 1 hit.
PS51509. PHOSPHAGEN_KINASE_N. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P17540-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MASIFSKLLT GRNASLLFAT MGTSVLTTGY LLNRQKVCAE VREQPRLFPP
60 70 80 90 100
SADYPDLRKH NNCMAECLTP AIYAKLRNKV TPNGYTLDQC IQTGVDNPGH
110 120 130 140 150
PFIKTVGMVA GDEESYEVFA DLFDPVIKLR HNGYDPRVMK HTTDLDASKI
160 170 180 190 200
TQGQFDEHYV LSSRVRTGRS IRGLSLPPAC TRAERREVEN VAITALEGLK
210 220 230 240 250
GDLAGRYYKL SEMTEQDQQR LIDDHFLFDK PVSPLLTCAG MARDWPDARG
260 270 280 290 300
IWHNYDKTFL IWINEEDHTR VISMEKGGNM KRVFERFCRG LKEVERLIQE
310 320 330 340 350
RGWEFMWNER LGYILTCPSN LGTGLRAGVH VRIPKLSKDP RFSKILENLR
360 370 380 390 400
LQKRGTGGVD TAAVADVYDI SNIDRIGRSE VELVQIVIDG VNYLVDCEKK
410
LERGQDIKVP PPLPQFGKK
Length:419
Mass (Da):47,504
Last modified:October 17, 2006 - v2
Checksum:i0CAF000074D99B6F
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti74 – 741A → S in AAA60561. (PubMed:2324105)Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J05401 mRNA. Translation: AAA60561.1.
CR450315 mRNA. Translation: CAG29311.1.
BC029140 mRNA. Translation: AAH29140.1.
CCDSiCCDS4053.1.
PIRiA35756.
RefSeqiNP_001093205.1. NM_001099735.1.
NP_001093206.1. NM_001099736.1.
NP_001816.2. NM_001825.2.
UniGeneiHs.80691.

Genome annotation databases

EnsembliENST00000254035; ENSP00000254035; ENSG00000131730.
ENST00000424301; ENSP00000404203; ENSG00000131730.
ENST00000437669; ENSP00000410289; ENSG00000131730.
GeneIDi1160.
KEGGihsa:1160.
UCSCiuc003khc.4. human.

Polymorphism databases

DMDMi116242603.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J05401 mRNA. Translation: AAA60561.1.
CR450315 mRNA. Translation: CAG29311.1.
BC029140 mRNA. Translation: AAH29140.1.
CCDSiCCDS4053.1.
PIRiA35756.
RefSeqiNP_001093205.1. NM_001099735.1.
NP_001093206.1. NM_001099736.1.
NP_001816.2. NM_001825.2.
UniGeneiHs.80691.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2GL6X-ray2.30A/B/C/D/E/F/G/H27-416[»]
ProteinModelPortaliP17540.
SMRiP17540. Positions 47-413.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107580. 8 interactions.
IntActiP17540. 6 interactions.
MINTiMINT-5004086.
STRINGi9606.ENSP00000254035.

Chemistry

DrugBankiDB00148. Creatine.

PTM databases

PhosphoSiteiP17540.

Polymorphism databases

DMDMi116242603.

2D gel databases

UCD-2DPAGEP17540.

Proteomic databases

MaxQBiP17540.
PaxDbiP17540.
PRIDEiP17540.

Protocols and materials databases

DNASUi1160.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000254035; ENSP00000254035; ENSG00000131730.
ENST00000424301; ENSP00000404203; ENSG00000131730.
ENST00000437669; ENSP00000410289; ENSG00000131730.
GeneIDi1160.
KEGGihsa:1160.
UCSCiuc003khc.4. human.

Organism-specific databases

CTDi1160.
GeneCardsiGC05P080530.
HGNCiHGNC:1996. CKMT2.
HPAiHPA051880.
MIMi123295. gene.
neXtProtiNX_P17540.
PharmGKBiPA26534.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG3869.
GeneTreeiENSGT00550000074561.
HOGENOMiHOG000232165.
HOVERGENiHBG001339.
InParanoidiP17540.
KOiK00933.
OMAiMTSIESK.
OrthoDBiEOG7XM2XW.
PhylomeDBiP17540.
TreeFamiTF314214.

Enzyme and pathway databases

BioCyciMetaCyc:HS05555-MONOMER.
ReactomeiREACT_813. Creatine metabolism.

Miscellaneous databases

EvolutionaryTraceiP17540.
GeneWikiiCKMT2.
GenomeRNAii1160.
NextBioi4814.
PROiP17540.
SOURCEiSearch...

Gene expression databases

BgeeiP17540.
CleanExiHS_CKMT2.
ExpressionAtlasiP17540. baseline and differential.
GenevestigatoriP17540.

Family and domain databases

Gene3Di1.10.135.10. 1 hit.
3.30.590.10. 1 hit.
InterProiIPR000749. ATP-guanido_PTrfase.
IPR022415. ATP-guanido_PTrfase_AS.
IPR022414. ATP-guanido_PTrfase_cat.
IPR022413. ATP-guanido_PTrfase_N.
IPR014746. Gln_synth/guanido_kin_cat_dom.
[Graphical view]
PANTHERiPTHR11547. PTHR11547. 1 hit.
PfamiPF00217. ATP-gua_Ptrans. 1 hit.
PF02807. ATP-gua_PtransN. 1 hit.
[Graphical view]
SUPFAMiSSF48034. SSF48034. 1 hit.
PROSITEiPS00112. PHOSPHAGEN_KINASE. 1 hit.
PS51510. PHOSPHAGEN_KINASE_C. 1 hit.
PS51509. PHOSPHAGEN_KINASE_N. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Separate nuclear genes encode sarcomere-specific and ubiquitous human mitochondrial creatine kinase isoenzymes."
    Haas R.C., Strauss A.W.
    J. Biol. Chem. 265:6921-6927(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Heart.
  2. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
    Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.
    Submitted (MAY-2004) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Brain.
  4. "Isolation and characterization of the gene and cDNA encoding human mitochondrial creatine kinase."
    Haas R.C., Korenfeld C., Zhang Z., Perryman B., Roman D., Strauss A.W.
    J. Biol. Chem. 264:2890-2897(1989) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 40-65.
  5. "Crystal structure of human sarcomeric mitochondrial creatine kinase."
    Structural genomics consortium (SGC)
    Submitted (APR-2006) to the PDB data bank
    Cited for: X-RAY CRYSTALLOGRAPHY (2.30 ANGSTROMS) OF 27-416 IN COMPLEX WITH ADP.

Entry informationi

Entry nameiKCRS_HUMAN
AccessioniPrimary (citable) accession number: P17540
Secondary accession number(s): Q6ICS8, Q8N1E1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: August 1, 1990
Last sequence update: October 17, 2006
Last modified: January 7, 2015
This is version 151 of the entry and version 2 of the sequence. [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

Miscellaneous

Mitochondrial creatine kinase binds cardiolipin.

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

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

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.