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Protein

Acylglycerol kinase, mitochondrial

Gene

AGK

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

Functioni

Lipid kinase that can phosphorylate both monoacylglycerol and diacylglycerol to form lysophosphatidic acid (LPA) and phosphatidic acid (PA), respectively. Does not phosphorylate sphingosine. Overexpression increases the formation and secretion of LPA, resulting in transactivation of EGFR and activation of the downstream MAPK signaling pathway, leading to increased cell growth.1 Publication

Catalytic activityi

ATP + acylglycerol = ADP + acyl-sn-glycerol 3-phosphate.
ATP + 1,2-diacyl-sn-glycerol = ADP + 1,2-diacyl-sn-glycerol 3-phosphate.

Cofactori

Mg2+1 Publication

Pathwayi: glycerolipid metabolism

This protein is involved in the pathway glycerolipid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway glycerolipid metabolism and in Lipid metabolism.

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Kinase, Transferase

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciZFISH:HS00180-MONOMER.
BRENDAi2.7.1.94. 2681.
ReactomeiR-HSA-6802952. Signaling by BRAF and RAF fusions.
R-HSA-75105. Fatty Acyl-CoA Biosynthesis.
SABIO-RKQ53H12.
UniPathwayiUPA00230.

Chemistry databases

SwissLipidsiSLP:000000638.

Names & Taxonomyi

Protein namesi
Recommended name:
Acylglycerol kinase, mitochondrial (EC:2.7.1.107, EC:2.7.1.94)
Short name:
hAGK
Alternative name(s):
Multiple substrate lipid kinase
Short name:
HsMuLK
Short name:
MuLK
Short name:
Multi-substrate lipid kinase
Gene namesi
Name:AGK
Synonyms:MULK
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

HGNCiHGNC:21869. AGK.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Mitochondrial DNA depletion syndrome 10 (MTDPS10)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive mitochondrial disorder characterized by congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, exercise intolerance, and lactic acidosis. Mental development is normal, but affected individuals may die early from cardiomyopathy.
See also OMIM:212350
Cataract 38 (CTRCT38)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn opacification of the crystalline lens of the eye becoming evident at birth. It frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function.
See also OMIM:614691

Keywords - Diseasei

Cardiomyopathy, Cataract

Organism-specific databases

DisGeNETi55750.
MalaCardsiAGK.
MIMi212350. phenotype.
614691. phenotype.
OpenTargetsiENSG00000006530.
Orphaneti1369. Congenital cataract - hypertrophic cardiomyopathy - mitochondrial myopathy.
91492. Non-syndromic congenital cataract.
PharmGKBiPA162375851.

Chemistry databases

ChEMBLiCHEMBL2417354.

Polymorphism and mutation databases

BioMutaiAGK.
DMDMi116248550.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 31MitochondrionSequence analysisAdd BLAST31
ChainiPRO_000025238032 – 422Acylglycerol kinase, mitochondrialAdd BLAST391

Proteomic databases

EPDiQ53H12.
MaxQBiQ53H12.
PaxDbiQ53H12.
PeptideAtlasiQ53H12.
PRIDEiQ53H12.

PTM databases

iPTMnetiQ53H12.
PhosphoSitePlusiQ53H12.
SwissPalmiQ53H12.

Expressioni

Tissue specificityi

Highly expressed in muscle, heart, kidney and brain.1 Publication

Gene expression databases

BgeeiENSG00000006530.
CleanExiHS_AGK.
ExpressionAtlasiQ53H12. baseline and differential.
GenevisibleiQ53H12. HS.

Organism-specific databases

HPAiHPA020959.
HPA053471.

Interactioni

Protein-protein interaction databases

BioGridi120868. 27 interactors.
IntActiQ53H12. 13 interactors.
MINTiMINT-1378485.
STRINGi9606.ENSP00000347581.

Structurei

3D structure databases

ProteinModelPortaliQ53H12.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini58 – 199DAGKcPROSITE-ProRule annotationAdd BLAST142

Sequence similaritiesi

Contains 1 DAGKc domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4435. Eukaryota.
COG1597. LUCA.
GeneTreeiENSGT00690000101761.
HOGENOMiHOG000252977.
HOVERGENiHBG080751.
InParanoidiQ53H12.
KOiK09881.
OMAiTKAAHFF.
OrthoDBiEOG091G06YD.
PhylomeDBiQ53H12.
TreeFamiTF320485.

Family and domain databases

Gene3Di3.40.50.10330. 1 hit.
InterProiIPR017438. ATP-NAD_kinase_dom_1.
IPR001206. Diacylglycerol_kinase_cat_dom.
IPR016064. NAD/diacylglycerol_kinase.
[Graphical view]
PfamiPF00781. DAGK_cat. 1 hit.
[Graphical view]
SMARTiSM00046. DAGKc. 1 hit.
[Graphical view]
SUPFAMiSSF111331. SSF111331. 2 hits.
PROSITEiPS50146. DAGK. 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: Q53H12-1) [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
MTVFFKTLRN HWKKTTAGLC LLTWGGHWLY GKHCDNLLRR AACQEAQVFG
60 70 80 90 100
NQLIPPNAQV KKATVFLNPA ACKGKARTLF EKNAAPILHL SGMDVTIVKT
110 120 130 140 150
DYEGQAKKLL ELMENTDVII VAGGDGTLQE VVTGVLRRTD EATFSKIPIG
160 170 180 190 200
FIPLGETSSL SHTLFAESGN KVQHITDATL AIVKGETVPL DVLQIKGEKE
210 220 230 240 250
QPVFAMTGLR WGSFRDAGVK VSKYWYLGPL KIKAAHFFST LKEWPQTHQA
260 270 280 290 300
SISYTGPTER PPNEPEETPV QRPSLYRRIL RRLASYWAQP QDALSQEVSP
310 320 330 340 350
EVWKDVQLST IELSITTRNN QLDPTSKEDF LNICIEPDTI SKGDFITIGS
360 370 380 390 400
RKVRNPKLHV EGTECLQASQ CTLLIPEGAG GSFSIDSEEY EAMPVEVKLL
410 420
PRKLQFFCDP RKREQMLTSP TQ
Length:422
Mass (Da):47,137
Last modified:October 17, 2006 - v2
Checksum:iF9D85658616B8970
GO
Isoform 2 (identifier: Q53H12-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     48-65: VFGNQLIPPNAQVKKATV → HYQDESRWEPTLSRTPGS
     66-422: Missing.

Note: No experimental confirmation available.
Show »
Length:65
Mass (Da):7,634
Checksum:i8F3133CE9A0B6D70
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti94D → V in BAD96489 (Ref. 3) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0278483V → M.Corresponds to variant rs10262855dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_02092548 – 65VFGNQ…KKATV → HYQDESRWEPTLSRTPGS in isoform 2. 1 PublicationAdd BLAST18
Alternative sequenceiVSP_02092666 – 422Missing in isoform 2. 1 PublicationAdd BLAST357

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ278150 mRNA. Translation: CAB93536.1.
AK001704 mRNA. Translation: BAA91848.1.
AK222769 mRNA. Translation: BAD96489.1.
AC004918 Genomic DNA. No translation available.
AC073878 Genomic DNA. No translation available.
AC099547 Genomic DNA. Translation: AAS07537.1.
BC009775 mRNA. Translation: AAH09775.1.
BC022777 mRNA. Translation: AAH22777.1.
CCDSiCCDS5865.1. [Q53H12-1]
RefSeqiNP_060708.1. NM_018238.3. [Q53H12-1]
XP_011514699.1. XM_011516397.2. [Q53H12-1]
UniGeneiHs.743318.

Genome annotation databases

EnsembliENST00000355413; ENSP00000347581; ENSG00000006530. [Q53H12-1]
ENST00000492693; ENSP00000418789; ENSG00000006530. [Q53H12-2]
ENST00000575872; ENSP00000458417; ENSG00000262327.
GeneIDi55750.
KEGGihsa:55750.
UCSCiuc003vwi.3. human. [Q53H12-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ278150 mRNA. Translation: CAB93536.1.
AK001704 mRNA. Translation: BAA91848.1.
AK222769 mRNA. Translation: BAD96489.1.
AC004918 Genomic DNA. No translation available.
AC073878 Genomic DNA. No translation available.
AC099547 Genomic DNA. Translation: AAS07537.1.
BC009775 mRNA. Translation: AAH09775.1.
BC022777 mRNA. Translation: AAH22777.1.
CCDSiCCDS5865.1. [Q53H12-1]
RefSeqiNP_060708.1. NM_018238.3. [Q53H12-1]
XP_011514699.1. XM_011516397.2. [Q53H12-1]
UniGeneiHs.743318.

3D structure databases

ProteinModelPortaliQ53H12.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120868. 27 interactors.
IntActiQ53H12. 13 interactors.
MINTiMINT-1378485.
STRINGi9606.ENSP00000347581.

Chemistry databases

ChEMBLiCHEMBL2417354.
SwissLipidsiSLP:000000638.

PTM databases

iPTMnetiQ53H12.
PhosphoSitePlusiQ53H12.
SwissPalmiQ53H12.

Polymorphism and mutation databases

BioMutaiAGK.
DMDMi116248550.

Proteomic databases

EPDiQ53H12.
MaxQBiQ53H12.
PaxDbiQ53H12.
PeptideAtlasiQ53H12.
PRIDEiQ53H12.

Protocols and materials databases

DNASUi55750.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000355413; ENSP00000347581; ENSG00000006530. [Q53H12-1]
ENST00000492693; ENSP00000418789; ENSG00000006530. [Q53H12-2]
ENST00000575872; ENSP00000458417; ENSG00000262327.
GeneIDi55750.
KEGGihsa:55750.
UCSCiuc003vwi.3. human. [Q53H12-1]

Organism-specific databases

CTDi55750.
DisGeNETi55750.
GeneCardsiAGK.
HGNCiHGNC:21869. AGK.
HPAiHPA020959.
HPA053471.
MalaCardsiAGK.
MIMi212350. phenotype.
610345. gene.
614691. phenotype.
neXtProtiNX_Q53H12.
OpenTargetsiENSG00000006530.
Orphaneti1369. Congenital cataract - hypertrophic cardiomyopathy - mitochondrial myopathy.
91492. Non-syndromic congenital cataract.
PharmGKBiPA162375851.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4435. Eukaryota.
COG1597. LUCA.
GeneTreeiENSGT00690000101761.
HOGENOMiHOG000252977.
HOVERGENiHBG080751.
InParanoidiQ53H12.
KOiK09881.
OMAiTKAAHFF.
OrthoDBiEOG091G06YD.
PhylomeDBiQ53H12.
TreeFamiTF320485.

Enzyme and pathway databases

UniPathwayiUPA00230.
BioCyciZFISH:HS00180-MONOMER.
BRENDAi2.7.1.94. 2681.
ReactomeiR-HSA-6802952. Signaling by BRAF and RAF fusions.
R-HSA-75105. Fatty Acyl-CoA Biosynthesis.
SABIO-RKQ53H12.

Miscellaneous databases

ChiTaRSiAGK. human.
GeneWikiiAGK_(gene).
GenomeRNAii55750.
PROiQ53H12.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000006530.
CleanExiHS_AGK.
ExpressionAtlasiQ53H12. baseline and differential.
GenevisibleiQ53H12. HS.

Family and domain databases

Gene3Di3.40.50.10330. 1 hit.
InterProiIPR017438. ATP-NAD_kinase_dom_1.
IPR001206. Diacylglycerol_kinase_cat_dom.
IPR016064. NAD/diacylglycerol_kinase.
[Graphical view]
PfamiPF00781. DAGK_cat. 1 hit.
[Graphical view]
SMARTiSM00046. DAGKc. 1 hit.
[Graphical view]
SUPFAMiSSF111331. SSF111331. 2 hits.
PROSITEiPS50146. DAGK. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiAGK_HUMAN
AccessioniPrimary (citable) accession number: Q53H12
Secondary accession number(s): Q75KN1, Q96GC3, Q9NP48
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 17, 2006
Last sequence update: October 17, 2006
Last modified: November 30, 2016
This is version 113 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

Overexpressed in prostate cancer, suggesting that it may play a role in initiation and progression of prostate cancer, processes in which LPA plays key roles.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. 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.