UniProtKB - P54819 (KAD2_HUMAN)
Adenylate kinase 2, mitochondrial
AK2
Functioni
Catalyzes the reversible transfer of the terminal phosphate group between ATP and AMP. Plays an important role in cellular energy homeostasis and in adenine nucleotide metabolism. Adenylate kinase activity is critical for regulation of the phosphate utilization and the AMP de novo biosynthesis pathways. Plays a key role in hematopoiesis.
UniRule annotation1 PublicationCatalytic activityi
- EC:2.7.4.3UniRule annotation
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 46 | AMPUniRule annotation | 1 | |
Binding sitei | 51 | AMPUniRule annotation | 1 | |
Binding sitei | 107 | AMPUniRule annotation | 1 | |
Binding sitei | 138 | ATP1 Publication | 1 | |
Binding sitei | 142 | ATPUniRule annotation | 1 | |
Binding sitei | 175 | AMPUniRule annotation1 Publication | 1 | |
Binding sitei | 186 | AMPUniRule annotation | 1 | |
Binding sitei | 214 | ATP; via carbonyl oxygenUniRule annotation1 Publication | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 25 – 30 | ATPUniRule annotation1 Publication | 6 | |
Nucleotide bindingi | 72 – 74 | AMPUniRule annotation | 3 | |
Nucleotide bindingi | 100 – 103 | AMPUniRule annotation | 4 | |
Nucleotide bindingi | 151 – 152 | ATPUniRule annotation1 Publication | 2 |
GO - Molecular functioni
- adenylate kinase activity Source: Reactome
- ATP binding Source: UniProtKB-KW
GO - Biological processi
- ADP biosynthetic process Source: UniProtKB-UniRule
- AMP metabolic process Source: UniProtKB-UniRule
- ATP metabolic process Source: UniProtKB-UniRule
- nucleobase-containing small molecule interconversion Source: Reactome
Keywordsi
Molecular function | Kinase, Transferase |
Ligand | ATP-binding, Nucleotide-binding |
Enzyme and pathway databases
PathwayCommonsi | P54819 |
Reactomei | R-HSA-499943, Interconversion of nucleotide di- and triphosphates |
SignaLinki | P54819 |
Names & Taxonomyi
Protein namesi | Recommended name: Adenylate kinase 2, mitochondrialUniRule annotation (EC:2.7.4.3UniRule annotation)Short name: AK 2UniRule annotation Alternative name(s): ATP-AMP transphosphorylase 2UniRule annotation ATP:AMP phosphotransferaseUniRule annotation Adenylate monophosphate kinaseUniRule annotation Cleaved into the following chain: Adenylate kinase 2, mitochondrial, N-terminally processedUniRule annotation |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:362, AK2 |
MIMi | 103020, gene |
neXtProti | NX_P54819 |
VEuPathDBi | HostDB:ENSG00000004455 |
Subcellular locationi
Mitochondrion
- Mitochondrion intermembrane space UniRule annotation
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
Mitochondrion
- mitochondrial intermembrane space Source: Reactome
- mitochondrion Source: GO_Central
Other locations
- cytoplasm Source: GO_Central
- sperm mitochondrial sheath Source: Ensembl
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Involvement in diseasei
Reticular dysgenesis (RDYS)2 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_054630 | 103 | R → W in RDYS. 1 PublicationCorresponds to variant dbSNP:rs267606648EnsemblClinVar. | 1 | |
Natural variantiVAR_054631 | 165 | D → G in RDYS. 1 PublicationCorresponds to variant dbSNP:rs267606643EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variant, SCIDOrganism-specific databases
DisGeNETi | 204 |
MalaCardsi | AK2 |
MIMi | 267500, phenotype |
OpenTargetsi | ENSG00000004455 |
Orphaneti | 33355, Reticular dysgenesis |
PharmGKBi | PA24656 |
Miscellaneous databases
Pharosi | P54819, Tbio |
Chemistry databases
ChEMBLi | CHEMBL4938 |
DrugBanki | DB00718, Adefovir dipivoxil DB01717, Bis(Adenosine)-5'-Pentaphosphate DB03366, Imidazole DB14126, Tenofovir DB09299, Tenofovir alafenamide DB00300, Tenofovir disoproxil |
Genetic variation databases
BioMutai | AK2 |
DMDMi | 1708596 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000423212 | 1 – 239 | Adenylate kinase 2, mitochondrialAdd BLAST | 239 | |
Initiator methioninei | Removed; alternateUniRule annotationCombined sources1 Publication | |||
ChainiPRO_0000158917 | 2 – 239 | Adenylate kinase 2, mitochondrial, N-terminally processedAdd BLAST | 238 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 1 | N-acetylmethionineCombined sources | 1 | |
Modified residuei | 4 | PhosphoserineCombined sources | 1 | |
Disulfide bondi | 42 ↔ 92 | UniRule annotation1 Publication | ||
Modified residuei | 58 | PhosphoserineCombined sources | 1 | |
Modified residuei | 62 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 93 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 133 | PhosphoserineCombined sources | 1 | |
Modified residuei | 181 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 195 | PhosphothreonineCombined sources | 1 |
Keywords - PTMi
Acetylation, Disulfide bond, PhosphoproteinProteomic databases
EPDi | P54819 |
jPOSTi | P54819 |
MassIVEi | P54819 |
MaxQBi | P54819 |
PaxDbi | P54819 |
PeptideAtlasi | P54819 |
PRIDEi | P54819 |
ProteomicsDBi | 56726 [P54819-1] 56727 [P54819-2] 56728 [P54819-3] 56729 [P54819-4] 56730 [P54819-5] 56731 [P54819-6] |
2D gel databases
OGPi | P54819 |
REPRODUCTION-2DPAGEi | IPI00218988 |
UCD-2DPAGEi | P54819 |
PTM databases
GlyGeni | P54819, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | P54819 |
MetOSitei | P54819 |
PhosphoSitePlusi | P54819 |
SwissPalmi | P54819 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000004455, Expressed in adult mammalian kidney and 228 other tissues |
ExpressionAtlasi | P54819, baseline and differential |
Genevisiblei | P54819, HS |
Organism-specific databases
HPAi | ENSG00000004455, Low tissue specificity |
Interactioni
Subunit structurei
Monomer.
UniRule annotationBinary interactionsi
P54819
Protein-protein interaction databases
BioGRIDi | 106707, 120 interactors |
CORUMi | P54819 |
IntActi | P54819, 62 interactors |
MINTi | P54819 |
STRINGi | 9606.ENSP00000346921 |
Chemistry databases
BindingDBi | P54819 |
Miscellaneous databases
RNActi | P54819, protein |
Structurei
Secondary structure
3D structure databases
AlphaFoldDBi | P54819 |
SMRi | P54819 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P54819 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 45 – 74 | NMPUniRule annotation1 PublicationAdd BLAST | 30 | |
Regioni | 141 – 178 | LIDUniRule annotation1 PublicationAdd BLAST | 38 | |
Regioni | 150 – 169 | DisorderedSequence analysisAdd BLAST | 20 |
Domaini
Sequence similaritiesi
Phylogenomic databases
eggNOGi | KOG3078, Eukaryota |
GeneTreei | ENSGT00940000154576 |
InParanoidi | P54819 |
OMAi | FHNRMRV |
PhylomeDBi | P54819 |
TreeFami | TF300896 |
Family and domain databases
CDDi | cd01428, ADK, 1 hit |
Gene3Di | 3.40.50.300, 1 hit |
HAMAPi | MF_00235, Adenylate_kinase_Adk, 1 hit MF_03168, Adenylate_kinase_AK2, 1 hit |
InterProi | View protein in InterPro IPR006259, Adenyl_kin_sub IPR000850, Adenylat/UMP-CMP_kin IPR033690, Adenylat_kinase_CS IPR007862, Adenylate_kinase_lid-dom IPR028587, AK2 IPR027417, P-loop_NTPase |
PANTHERi | PTHR23359, PTHR23359, 1 hit |
Pfami | View protein in Pfam PF05191, ADK_lid, 1 hit |
PRINTSi | PR00094, ADENYLTKNASE |
SUPFAMi | SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR01351, adk, 1 hit |
PROSITEi | View protein in PROSITE PS00113, ADENYLATE_KINASE, 1 hit |
s (6+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 6 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 6 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MAPSVPAAEP EYPKGIRAVL LGPPGAGKGT QAPRLAENFC VCHLATGDML
60 70 80 90 100
RAMVASGSEL GKKLKATMDA GKLVSDEMVV ELIEKNLETP LCKNGFLLDG
110 120 130 140 150
FPRTVRQAEM LDDLMEKRKE KLDSVIEFSI PDSLLIRRIT GRLIHPKSGR
160 170 180 190 200
SYHEEFNPPK EPMKDDITGE PLIRRSDDNE KALKIRLQAY HTQTTPLIEY
210 220 230
YRKRGIHSAI DASQTPDVVF ASILAAFSKA TCKDLVMFI
Computationally mapped potential isoform sequencesi
There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketF8W1A4 | F8W1A4_HUMAN | Adenylate kinase 2, mitochondrial | AK2 | 232 | Annotation score: | ||
F8VY04 | F8VY04_HUMAN | Nucleoside-diphosphate kinase | AK2 | 190 | Annotation score: | ||
G3V213 | G3V213_HUMAN | Nucleoside-diphosphate kinase | AK2 hCG_2032090 | 133 | Annotation score: | ||
A0A5K1VW67 | A0A5K1VW67_HUMAN | Nucleoside-diphosphate kinase | AK2 | 197 | Annotation score: | ||
F8VZG5 | F8VZG5_HUMAN | Nucleoside-diphosphate kinase | AK2 | 162 | Annotation score: | ||
A0A5F9ZHP2 | A0A5F9ZHP2_HUMAN | Nucleoside-diphosphate kinase | AK2 | 162 | Annotation score: | ||
F8VPP1 | F8VPP1_HUMAN | Adenylate kinase 2, mitochondrial | AK2 | 42 | Annotation score: |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_054630 | 103 | R → W in RDYS. 1 PublicationCorresponds to variant dbSNP:rs267606648EnsemblClinVar. | 1 | |
Natural variantiVAR_054631 | 165 | D → G in RDYS. 1 PublicationCorresponds to variant dbSNP:rs267606643EnsemblClinVar. | 1 | |
Natural variantiVAR_050032 | 209 | A → T. Corresponds to variant dbSNP:rs12116440EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_002792 | 1 – 48 | Missing in isoform 4 and isoform 6. 2 PublicationsAdd BLAST | 48 | |
Alternative sequenceiVSP_036503 | 135 – 142 | Missing in isoform 5. 1 Publication | 8 | |
Alternative sequenceiVSP_002793 | 177 – 178 | DD → GL in isoform 4. 1 Publication | 2 | |
Alternative sequenceiVSP_002791 | 178 – 239 | DNEKA…LVMFI → IGQAKRSFLRLAKISFDVLI KKALA in isoform 3. 1 PublicationAdd BLAST | 62 | |
Alternative sequenceiVSP_002794 | 179 – 239 | Missing in isoform 4. 1 PublicationAdd BLAST | 61 | |
Alternative sequenceiVSP_002790 | 232 – 239 | CKDLVMFI → S in isoform 2 and isoform 5. 5 Publications | 8 |
Sequence databases
Genome annotation databases
Ensembli | ENST00000373449.7; ENSP00000362548.2; ENSG00000004455.17 [P54819-2] ENST00000672715.1; ENSP00000499935.1; ENSG00000004455.17 |
GeneIDi | 204 |
KEGGi | hsa:204 |
MANE-Selecti | ENST00000672715.1; ENSP00000499935.1; NM_001625.4; NP_001616.1 |
UCSCi | uc001bwo.3, human [P54819-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | U39945 mRNA Translation: AAC52061.1 U84371 mRNA Translation: AAB41790.1 U54645 mRNA Translation: AAC13881.1 AB005621 mRNA Translation: BAC16747.1 AB005622 mRNA Translation: BAC16748.1 AY080899 mRNA Translation: AAL87027.1 AY080900 mRNA Translation: AAL87028.1 AK291676 mRNA Translation: BAF84365.1 AK295105 mRNA Translation: BAG58139.1 AK296863 mRNA Translation: BAG59426.1 AB451267 mRNA Translation: BAG70081.1 AB451394 mRNA Translation: BAG70208.1 AL020995 Genomic DNA No translation available. CH471059 Genomic DNA Translation: EAX07484.1 CH471059 Genomic DNA Translation: EAX07486.1 BC009405 mRNA Translation: AAH09405.1 BC070127 mRNA Translation: AAH70127.1 BC090040 mRNA Translation: AAH90040.1 |
CCDSi | CCDS373.1 [P54819-2] CCDS374.1 [P54819-1] |
PIRi | G02248 JC5893 |
RefSeqi | NP_001186128.1, NM_001199199.1 [P54819-5] NP_001306068.1, NM_001319139.1 NP_001306069.1, NM_001319140.1 [P54819-6] NP_001616.1, NM_001625.3 [P54819-1] NP_037543.1, NM_013411.4 [P54819-2] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
2C9Y | X-ray | 2.10 | A | 1-239 | [»] | |
AlphaFoldDBi | P54819 | |||||
SMRi | P54819 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 106707, 120 interactors |
CORUMi | P54819 |
IntActi | P54819, 62 interactors |
MINTi | P54819 |
STRINGi | 9606.ENSP00000346921 |
Chemistry databases
BindingDBi | P54819 |
ChEMBLi | CHEMBL4938 |
DrugBanki | DB00718, Adefovir dipivoxil DB01717, Bis(Adenosine)-5'-Pentaphosphate DB03366, Imidazole DB14126, Tenofovir DB09299, Tenofovir alafenamide DB00300, Tenofovir disoproxil |
PTM databases
GlyGeni | P54819, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | P54819 |
MetOSitei | P54819 |
PhosphoSitePlusi | P54819 |
SwissPalmi | P54819 |
Genetic variation databases
BioMutai | AK2 |
DMDMi | 1708596 |
2D gel databases
OGPi | P54819 |
REPRODUCTION-2DPAGEi | IPI00218988 |
UCD-2DPAGEi | P54819 |
Proteomic databases
EPDi | P54819 |
jPOSTi | P54819 |
MassIVEi | P54819 |
MaxQBi | P54819 |
PaxDbi | P54819 |
PeptideAtlasi | P54819 |
PRIDEi | P54819 |
ProteomicsDBi | 56726 [P54819-1] 56727 [P54819-2] 56728 [P54819-3] 56729 [P54819-4] 56730 [P54819-5] 56731 [P54819-6] |
Protocols and materials databases
Antibodypediai | 17047, 461 antibodies from 33 providers |
DNASUi | 204 |
Genome annotation databases
Ensembli | ENST00000373449.7; ENSP00000362548.2; ENSG00000004455.17 [P54819-2] ENST00000672715.1; ENSP00000499935.1; ENSG00000004455.17 |
GeneIDi | 204 |
KEGGi | hsa:204 |
MANE-Selecti | ENST00000672715.1; ENSP00000499935.1; NM_001625.4; NP_001616.1 |
UCSCi | uc001bwo.3, human [P54819-1] |
Organism-specific databases
CTDi | 204 |
DisGeNETi | 204 |
GeneCardsi | AK2 |
HGNCi | HGNC:362, AK2 |
HPAi | ENSG00000004455, Low tissue specificity |
MalaCardsi | AK2 |
MIMi | 103020, gene 267500, phenotype |
neXtProti | NX_P54819 |
OpenTargetsi | ENSG00000004455 |
Orphaneti | 33355, Reticular dysgenesis |
PharmGKBi | PA24656 |
VEuPathDBi | HostDB:ENSG00000004455 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG3078, Eukaryota |
GeneTreei | ENSGT00940000154576 |
InParanoidi | P54819 |
OMAi | FHNRMRV |
PhylomeDBi | P54819 |
TreeFami | TF300896 |
Enzyme and pathway databases
PathwayCommonsi | P54819 |
Reactomei | R-HSA-499943, Interconversion of nucleotide di- and triphosphates |
SignaLinki | P54819 |
Miscellaneous databases
BioGRID-ORCSi | 204, 94 hits in 1073 CRISPR screens |
ChiTaRSi | AK2, human |
EvolutionaryTracei | P54819 |
GeneWikii | AK2 |
GenomeRNAii | 204 |
Pharosi | P54819, Tbio |
PROi | PR:P54819 |
RNActi | P54819, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000004455, Expressed in adult mammalian kidney and 228 other tissues |
ExpressionAtlasi | P54819, baseline and differential |
Genevisiblei | P54819, HS |
Family and domain databases
CDDi | cd01428, ADK, 1 hit |
Gene3Di | 3.40.50.300, 1 hit |
HAMAPi | MF_00235, Adenylate_kinase_Adk, 1 hit MF_03168, Adenylate_kinase_AK2, 1 hit |
InterProi | View protein in InterPro IPR006259, Adenyl_kin_sub IPR000850, Adenylat/UMP-CMP_kin IPR033690, Adenylat_kinase_CS IPR007862, Adenylate_kinase_lid-dom IPR028587, AK2 IPR027417, P-loop_NTPase |
PANTHERi | PTHR23359, PTHR23359, 1 hit |
Pfami | View protein in Pfam PF05191, ADK_lid, 1 hit |
PRINTSi | PR00094, ADENYLTKNASE |
SUPFAMi | SSF52540, SSF52540, 1 hit |
TIGRFAMsi | TIGR01351, adk, 1 hit |
PROSITEi | View protein in PROSITE PS00113, ADENYLATE_KINASE, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | KAD2_HUMAN | |
Accessioni | P54819Primary (citable) accession number: P54819 Secondary accession number(s): A8K6L1 Q8TCY3 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 1, 1996 |
Last sequence update: | January 23, 2007 | |
Last modified: | May 25, 2022 | |
This is version 213 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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, Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families