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Protein

Creatine kinase U-type, mitochondrial

Gene

CKMT1A

more
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)DescriptionActionsGraphical viewLength
Binding sitei224ATPPROSITE-ProRule annotation1
Binding sitei269ATPPROSITE-ProRule annotation1
Binding sitei325ATPPROSITE-ProRule annotation1
Binding sitei368ATPPROSITE-ProRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi161 – 165ATPPROSITE-ProRule annotation5
Nucleotide bindingi353 – 358ATPPROSITE-ProRule annotation6

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Kinase, Transferase

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS09820-MONOMER.
ZFISH:HS09820-MONOMER.
BRENDAi2.7.3.2. 2681.
ReactomeiR-HSA-71288. Creatine metabolism.

Names & Taxonomyi

Protein namesi
Recommended name:
Creatine kinase U-type, mitochondrial (EC:2.7.3.2)
Alternative name(s):
Acidic-type mitochondrial creatine kinase
Short name:
Mia-CK
Ubiquitous mitochondrial creatine kinase
Short name:
U-MtCK
Gene namesi
Name:CKMT1A
Synonyms:CKMT
AND
Name:CKMT1B
Synonyms:CKMT
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 15

Organism-specific databases

HGNCiHGNC:31736. CKMT1A.
HGNC:1995. CKMT1B.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • mitochondrial inner membrane Source: Reactome
  • mitochondrion Source: ProtInc
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi1159.
548596.
OpenTargetsiENSG00000223572.
ENSG00000237289.
PharmGKBiPA142672108.

Chemistry databases

DrugBankiDB00148. Creatine.

Polymorphism and mutation databases

BioMutaiCKMT1A.
DMDMi125315.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 39MitochondrionAdd BLAST39
ChainiPRO_000001659040 – 417Creatine kinase U-type, mitochondrialAdd BLAST378

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei151PhosphoserineBy similarity1
Modified residuei196PhosphoserineBy similarity1
Modified residuei213PhosphothreonineBy similarity1
Modified residuei232PhosphoserineBy similarity1
Modified residuei355PhosphothreonineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP12532.
MaxQBiP12532.
PaxDbiP12532.
PeptideAtlasiP12532.
PRIDEiP12532.

2D gel databases

UCD-2DPAGEP12532.

PTM databases

iPTMnetiP12532.
PhosphoSitePlusiP12532.
SwissPalmiP12532.

Expressioni

Gene expression databases

BgeeiENSG00000223572.
CleanExiHS_CKMT1A.
HS_CKMT1B.
ExpressionAtlasiP12532. baseline and differential.
GenevisibleiP12532. HS.

Organism-specific databases

HPAiHPA043491.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi107579. 20 interactors.
139227. 17 interactors.
IntActiP12532. 9 interactors.
MINTiMINT-3304503.
STRINGi9606.ENSP00000406577.

Structurei

Secondary structure

1417
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi49 – 52Combined sources4
Helixi62 – 66Combined sources5
Helixi69 – 75Combined sources7
Helixi86 – 95Combined sources10
Beta strandi100 – 102Combined sources3
Helixi114 – 117Combined sources4
Helixi119 – 129Combined sources11
Turni130 – 132Combined sources3
Turni135 – 137Combined sources3
Helixi146 – 148Combined sources3
Turni156 – 158Combined sources3
Beta strandi159 – 168Combined sources10
Turni176 – 178Combined sources3
Helixi181 – 195Combined sources15
Helixi200 – 202Combined sources3
Beta strandi204 – 208Combined sources5
Helixi209 – 211Combined sources3
Helixi214 – 222Combined sources9
Helixi233 – 236Combined sources4
Turni237 – 247Combined sources11
Beta strandi249 – 253Combined sources5
Beta strandi258 – 277Combined sources20
Helixi279 – 298Combined sources20
Turni299 – 301Combined sources3
Turni308 – 310Combined sources3
Helixi317 – 319Combined sources3
Beta strandi325 – 331Combined sources7
Helixi333 – 337Combined sources5
Helixi341 – 348Combined sources8
Beta strandi350 – 353Combined sources4
Turni359 – 361Combined sources3
Beta strandi364 – 372Combined sources9
Beta strandi375 – 377Combined sources3
Helixi379 – 400Combined sources22
Turni401 – 403Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1QK1X-ray2.70A/B/C/D/E/F/G/H39-417[»]
ProteinModelPortaliP12532.
SMRiP12532.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP12532.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini45 – 131Phosphagen kinase N-terminalPROSITE-ProRule annotationAdd BLAST87
Domaini158 – 400Phosphagen kinase C-terminalPROSITE-ProRule annotationAdd BLAST243

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni40 – 64Cardiolipin-bindingBy similarityAdd BLAST25

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

eggNOGiKOG3581. Eukaryota.
COG3869. LUCA.
GeneTreeiENSGT00550000074561.
HOGENOMiHOG000232165.
HOVERGENiHBG001339.
InParanoidiP12532.
KOiK00933.
OMAiERHNGYN.
OrthoDBiEOG091G0HZ0.
PhylomeDBiP12532.
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]

Sequences (2)i

Sequence statusi: Complete.

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

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

Isoform 1 (identifier: P12532-1) [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
MAGPFSRLLS ARPGLRLLAL AGAGSLAAGF LLRPEPVRAA SERRRLYPPS
60 70 80 90 100
AEYPDLRKHN NCMASHLTPA VYARLCDKTT PTGWTLDQCI QTGVDNPGHP
110 120 130 140 150
FIKTVGMVAG DEETYEVFAD LFDPVIQERH NGYDPRTMKH TTDLDASKIR
160 170 180 190 200
SGYFDERYVL SSRVRTGRSI RGLSLPPACT RAERREVERV VVDALSGLKG
210 220 230 240 250
DLAGRYYRLS EMTEAEQQQL IDDHFLFDKP VSPLLTAAGM ARDWPDARGI
260 270 280 290 300
WHNNEKSFLI WVNEEDHTRV ISMEKGGNMK RVFERFCRGL KEVERLIQER
310 320 330 340 350
GWEFMWNERL GYILTCPSNL GTGLRAGVHI KLPLLSKDSR FPKILENLRL
360 370 380 390 400
QKRGTGGVDT AATGGVFDIS NLDRLGKSEV ELVQLVIDGV NYLIDCERRL
410
ERGQDIRIPT PVIHTKH
Length:417
Mass (Da):47,037
Last modified:October 1, 1989 - v1
Checksum:i274DAC2E9A8AD882
GO
Isoform 2 (identifier: P12532-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     50-50: S → SQTWPTGQLPGNCTRSRRLCPPSMVTGYPLPS

Show »
Length:448
Mass (Da):50,421
Checksum:i460CD2E9E9D6A0E2
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti176P → L in BAG58600 (PubMed:14702039).Curated1
Sequence conflicti401E → G in BAD97085 (Ref. 4) Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_03804550S → SQTWPTGQLPGNCTRSRRLC PPSMVTGYPLPS in isoform 2. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04469 Genomic DNA. Translation: AAA98744.1.
BT006628 mRNA. Translation: AAP35274.1.
AK295776 mRNA. Translation: BAG58600.1.
AK223365 mRNA. Translation: BAD97085.1.
AK316124 mRNA. Translation: BAH14495.1.
AK316319 mRNA. Translation: BAH14690.1.
BC001926 mRNA. Translation: AAH01926.1.
BC006467 mRNA. Translation: AAH06467.1.
BC108652 mRNA. Translation: AAI08653.1.
BC121001 mRNA. Translation: AAI21002.1.
BC121002 mRNA. Translation: AAI21003.1.
CCDSiCCDS10097.1. [P12532-1]
CCDS32217.1. [P12532-1]
PIRiA31431. A30789.
RefSeqiNP_001015001.1. NM_001015001.2. [P12532-1]
NP_001308855.1. NM_001321926.1. [P12532-1]
NP_001308856.1. NM_001321927.1. [P12532-2]
NP_001308857.1. NM_001321928.1. [P12532-2]
NP_066270.1. NM_020990.4. [P12532-1]
XP_011519496.1. XM_011521194.1. [P12532-2]
XP_011519497.1. XM_011521195.2. [P12532-2]
XP_011519498.1. XM_011521196.1. [P12532-2]
XP_011519499.1. XM_011521197.2. [P12532-1]
XP_016877858.1. XM_017022369.1. [P12532-2]
XP_016877859.1. XM_017022370.1. [P12532-2]
UniGeneiHs.654988.
Hs.741420.

Genome annotation databases

EnsembliENST00000300283; ENSP00000300283; ENSG00000237289. [P12532-1]
ENST00000413453; ENSP00000406577; ENSG00000223572. [P12532-1]
ENST00000434505; ENSP00000413165; ENSG00000223572. [P12532-1]
ENST00000441322; ENSP00000413255; ENSG00000237289. [P12532-1]
GeneIDi1159.
548596.
KEGGihsa:1159.
hsa:548596.
UCSCiuc001zsc.3. human. [P12532-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04469 Genomic DNA. Translation: AAA98744.1.
BT006628 mRNA. Translation: AAP35274.1.
AK295776 mRNA. Translation: BAG58600.1.
AK223365 mRNA. Translation: BAD97085.1.
AK316124 mRNA. Translation: BAH14495.1.
AK316319 mRNA. Translation: BAH14690.1.
BC001926 mRNA. Translation: AAH01926.1.
BC006467 mRNA. Translation: AAH06467.1.
BC108652 mRNA. Translation: AAI08653.1.
BC121001 mRNA. Translation: AAI21002.1.
BC121002 mRNA. Translation: AAI21003.1.
CCDSiCCDS10097.1. [P12532-1]
CCDS32217.1. [P12532-1]
PIRiA31431. A30789.
RefSeqiNP_001015001.1. NM_001015001.2. [P12532-1]
NP_001308855.1. NM_001321926.1. [P12532-1]
NP_001308856.1. NM_001321927.1. [P12532-2]
NP_001308857.1. NM_001321928.1. [P12532-2]
NP_066270.1. NM_020990.4. [P12532-1]
XP_011519496.1. XM_011521194.1. [P12532-2]
XP_011519497.1. XM_011521195.2. [P12532-2]
XP_011519498.1. XM_011521196.1. [P12532-2]
XP_011519499.1. XM_011521197.2. [P12532-1]
XP_016877858.1. XM_017022369.1. [P12532-2]
XP_016877859.1. XM_017022370.1. [P12532-2]
UniGeneiHs.654988.
Hs.741420.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1QK1X-ray2.70A/B/C/D/E/F/G/H39-417[»]
ProteinModelPortaliP12532.
SMRiP12532.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107579. 20 interactors.
139227. 17 interactors.
IntActiP12532. 9 interactors.
MINTiMINT-3304503.
STRINGi9606.ENSP00000406577.

Chemistry databases

DrugBankiDB00148. Creatine.

PTM databases

iPTMnetiP12532.
PhosphoSitePlusiP12532.
SwissPalmiP12532.

Polymorphism and mutation databases

BioMutaiCKMT1A.
DMDMi125315.

2D gel databases

UCD-2DPAGEP12532.

Proteomic databases

EPDiP12532.
MaxQBiP12532.
PaxDbiP12532.
PeptideAtlasiP12532.
PRIDEiP12532.

Protocols and materials databases

DNASUi1159.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000300283; ENSP00000300283; ENSG00000237289. [P12532-1]
ENST00000413453; ENSP00000406577; ENSG00000223572. [P12532-1]
ENST00000434505; ENSP00000413165; ENSG00000223572. [P12532-1]
ENST00000441322; ENSP00000413255; ENSG00000237289. [P12532-1]
GeneIDi1159.
548596.
KEGGihsa:1159.
hsa:548596.
UCSCiuc001zsc.3. human. [P12532-1]

Organism-specific databases

CTDi1159.
548596.
DisGeNETi1159.
548596.
GeneCardsiCKMT1A.
CKMT1B.
HGNCiHGNC:31736. CKMT1A.
HGNC:1995. CKMT1B.
HPAiHPA043491.
MIMi123290. gene.
613415. gene.
neXtProtiNX_P12532.
OpenTargetsiENSG00000223572.
ENSG00000237289.
PharmGKBiPA142672108.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3581. Eukaryota.
COG3869. LUCA.
GeneTreeiENSGT00550000074561.
HOGENOMiHOG000232165.
HOVERGENiHBG001339.
InParanoidiP12532.
KOiK00933.
OMAiERHNGYN.
OrthoDBiEOG091G0HZ0.
PhylomeDBiP12532.
TreeFamiTF314214.

Enzyme and pathway databases

BioCyciMetaCyc:HS09820-MONOMER.
ZFISH:HS09820-MONOMER.
BRENDAi2.7.3.2. 2681.
ReactomeiR-HSA-71288. Creatine metabolism.

Miscellaneous databases

ChiTaRSiCKMT1B. human.
EvolutionaryTraceiP12532.
GeneWikiiCKMT1B.
PROiP12532.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000223572.
CleanExiHS_CKMT1A.
HS_CKMT1B.
ExpressionAtlasiP12532. baseline and differential.
GenevisibleiP12532. HS.

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...

Entry informationi

Entry nameiKCRU_HUMAN
AccessioniPrimary (citable) accession number: P12532
Secondary accession number(s): B4DIT8
, B7ZA09, Q0VAM3, Q32NF6, Q53FC4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1989
Last sequence update: October 1, 1989
Last modified: November 30, 2016
This is version 182 of the entry and version 1 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, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: 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

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 one 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.