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Protein

Creatine kinase U-type, mitochondrial

Gene

CKMT1A

more
Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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.

Miscellaneous

Mitochondrial creatine kinase binds cardiolipin.

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

Keywordsi

Molecular functionKinase, Transferase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc: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

EuPathDBiHostDB:ENSG00000223572.9
HostDB:ENSG00000237289.9
HGNCiHGNC:31736 CKMT1A
HGNC:1995 CKMT1B
MIMi123290 gene
613415 gene
neXtProtiNX_P12532

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

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
ProteomicsDBi52856
52857 [P12532-2]

2D gel databases

UCD-2DPAGEiP12532

PTM databases

iPTMnetiP12532
PhosphoSitePlusiP12532
SwissPalmiP12532

Expressioni

Gene expression databases

BgeeiENSG00000223572 Expressed in 86 organ(s), highest expression level in right hemisphere of cerebellum
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

Binary interactionsi

Protein-protein interaction databases

BioGridi107579, 23 interactors
139227, 20 interactors
IntActiP12532, 21 interactors
MINTiP12532
STRINGi9606.ENSP00000406577

Structurei

Secondary structure

1417
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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

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
InterProiView protein in InterPro
IPR000749 ATP-guanido_PTrfase
IPR022415 ATP-guanido_PTrfase_AS
IPR022414 ATP-guanido_PTrfase_cat
IPR022413 ATP-guanido_PTrfase_N
IPR036802 ATP-guanido_PTrfase_N_sf
IPR014746 Gln_synth/guanido_kin_cat_dom
PANTHERiPTHR11547 PTHR11547, 1 hit
PfamiView protein in Pfam
PF00217 ATP-gua_Ptrans, 1 hit
PF02807 ATP-gua_PtransN, 1 hit
SUPFAMiSSF48034 SSF48034, 1 hit
SSF55931 SSF55931, 1 hit
PROSITEiView protein in PROSITE
PS00112 PHOSPHAGEN_KINASE, 1 hit
PS51510 PHOSPHAGEN_KINASE_C, 1 hit
PS51509 PHOSPHAGEN_KINASE_N, 1 hit

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

This entry has 2 described isoforms and 10 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 10 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F8WCN3F8WCN3_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1A CKMT1B
298Annotation score:
C9JSQ1C9JSQ1_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
241Annotation score:
C9J6W7C9J6W7_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
203Annotation score:
C9JT96C9JT96_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1A
221Annotation score:
C9J995C9J995_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
129Annotation score:
C9JJX8C9JJX8_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
103Annotation score:
C9J8F6C9J8F6_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1A
148Annotation score:
C9JFS8C9JFS8_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
96Annotation score:
F8WDP9F8WDP9_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1A
69Annotation score:
F8WFC7F8WFC7_HUMAN
Creatine kinase U-type, mitochondri...
CKMT1B
58Annotation score:

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

Similar proteinsi

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, 23 interactors
139227, 20 interactors
IntActiP12532, 21 interactors
MINTiP12532
STRINGi9606.ENSP00000406577

Chemistry databases

DrugBankiDB00148 Creatine

PTM databases

iPTMnetiP12532
PhosphoSitePlusiP12532
SwissPalmiP12532

Polymorphism and mutation databases

BioMutaiCKMT1A
DMDMi125315

2D gel databases

UCD-2DPAGEiP12532

Proteomic databases

EPDiP12532
MaxQBiP12532
PaxDbiP12532
PeptideAtlasiP12532
PRIDEiP12532
ProteomicsDBi52856
52857 [P12532-2]

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
EuPathDBiHostDB:ENSG00000223572.9
HostDB:ENSG00000237289.9
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
BRENDAi2.7.3.2 2681
ReactomeiR-HSA-71288 Creatine metabolism

Miscellaneous databases

ChiTaRSiCKMT1B human
EvolutionaryTraceiP12532
GeneWikiiCKMT1B
PROiPR:P12532
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000223572 Expressed in 86 organ(s), highest expression level in right hemisphere of cerebellum
CleanExiHS_CKMT1A
HS_CKMT1B
ExpressionAtlasiP12532 baseline and differential
GenevisibleiP12532 HS

Family and domain databases

Gene3Di1.10.135.10, 1 hit
InterProiView protein in InterPro
IPR000749 ATP-guanido_PTrfase
IPR022415 ATP-guanido_PTrfase_AS
IPR022414 ATP-guanido_PTrfase_cat
IPR022413 ATP-guanido_PTrfase_N
IPR036802 ATP-guanido_PTrfase_N_sf
IPR014746 Gln_synth/guanido_kin_cat_dom
PANTHERiPTHR11547 PTHR11547, 1 hit
PfamiView protein in Pfam
PF00217 ATP-gua_Ptrans, 1 hit
PF02807 ATP-gua_PtransN, 1 hit
SUPFAMiSSF48034 SSF48034, 1 hit
SSF55931 SSF55931, 1 hit
PROSITEiView protein in PROSITE
PS00112 PHOSPHAGEN_KINASE, 1 hit
PS51510 PHOSPHAGEN_KINASE_C, 1 hit
PS51509 PHOSPHAGEN_KINASE_N, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiKCRU_HUMAN
AccessioniPrimary (citable) accession number: P12532
Secondary accession number(s): B4DIT8
, B7ZA09, Q0VAM3, Q32NF6, Q53FC4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1989
Last sequence update: October 1, 1989
Last modified: November 7, 2018
This is version 197 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 15
    Human chromosome 15: entries, gene names and cross-references to MIM
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