acylglycerol kinase


Gene Symbol: acylglycerol kinase
Description: acylglycerol kinase
Alias: CATC5, CTRCT38, MTDPS10, MULK, acylglycerol kinase, mitochondrial, hAGK, hsMuLK, multi-substrate lipid kinase, multiple substrate lipid kinase
Species: human
Products:     acylglycerol kinase

Top Publications

  1. Waggoner D, Johnson L, Mann P, Morris V, Guastella J, Bajjalieh S. MuLK, a eukaryotic multi-substrate lipid kinase. J Biol Chem. 2004;279:38228-35 pubmed
    ..This enzyme, which we term MuLK for multi-substrate lipid kinase, does not belong to a previously described lipid kinase family...
  2. Chen X, Ying Z, Lin X, Lin H, Wu J, Li M, et al. Acylglycerol kinase augments JAK2/STAT3 signaling in esophageal squamous cells. J Clin Invest. 2013;123:2576-89 pubmed publisher
    ..These findings uncover a mechanism for constitutive activation of JAK2/STAT3 signaling in solid tumors and may represent a prognostic biomarker and therapeutic target. ..
  3. Siriwardena K, Mackay N, Levandovskiy V, Blaser S, Raiman J, Kantor P, et al. Mitochondrial citrate synthase crystals: novel finding in Sengers syndrome caused by acylglycerol kinase (AGK) mutations. Mol Genet Metab. 2013;108:40-50 pubmed publisher
    We report on two families with Sengers syndrome and mutations in the acylglycerol kinase gene (AGK)...
  4. Aldahmesh M, Khan A, Mohamed J, Alghamdi M, Alkuraya F. Identification of a truncation mutation of acylglycerol kinase (AGK) gene in a novel autosomal recessive cataract locus. Hum Mutat. 2012;33:960-2 pubmed publisher
    ..1. Exome sequencing revealed a splice-site mutation in AGK, encoding acylglycerol kinase, which we confirm led to aberrant splicing and predicted premature truncation...
  5. Mayr J, Haack T, Graf E, Zimmermann F, Wieland T, Haberberger B, et al. Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome. Am J Hum Genet. 2012;90:314-20 pubmed publisher
    ..typical symptoms of Sengers syndrome, discovered two nonsense mutations in the gene encoding mitochondrial acylglycerol kinase (AGK)...
  6. Bektas M, Payne S, Liu H, Goparaju S, Milstien S, Spiegel S. A novel acylglycerol kinase that produces lysophosphatidic acid modulates cross talk with EGFR in prostate cancer cells. J Cell Biol. 2005;169:801-11 pubmed
    ..Here, we report characterization of a novel lipid kinase, designated acylglycerol kinase (AGK), that phosphorylates monoacylglycerol and diacylglycerol to form LPA and PA, respectively...
  7. Wang X, Lin C, Zhao X, Liu A, Zhu J, Li X, et al. Acylglycerol kinase promotes cell proliferation and tumorigenicity in breast cancer via suppression of the FOXO1 transcription factor. Mol Cancer. 2014;13:106 pubmed publisher
    b>Acylglycerol kinase (AGK) is reported to be overexpressed in multiple cancers. The clinical significance and biological role of AGK in breast cancer, however, remain to be established...
  8. Epand R, Shulga Y, Timmons H, Perri A, Belani J, Perinpanathan K, et al. Substrate chirality and specificity of diacylglycerol kinases and the multisubstrate lipid kinase. Biochemistry. 2007;46:14225-31 pubmed
    ..In comparison, a multiple lipid kinase, MuLK, shows much less stereospecificity, phosphorylating 1,2-dioleoylglycerol only approximately 2-3 times more rapidly ..
  9. Nouh M, Wu X, Okazoe H, Tsunemori H, Haba R, Abou Zeid A, et al. Expression of autotaxin and acylglycerol kinase in prostate cancer: association with cancer development and progression. Cancer Sci. 2009;100:1631-8 pubmed publisher
    ..This study was conducted to analyze expression levels of LPA-producing enzymes, autotaxin (ATX) and acylglycerol kinase (AGK), in prostate cancer with relevance to clinicopathological parameters...

More Information


  1. Vojnic M, Kubota D, Kurzatkowski C, Offin M, Suzawa K, Benayed R, et al. Acquired BRAF Rearrangements Induce Secondary Resistance to EGFR therapy in EGFR-Mutated Lung Cancers. J Thorac Oncol. 2019;14:802-815 pubmed publisher
    ..We identified four patients (2.3%) with a BRAF fusion (three with acylglycerol kinase gene (AGK)/BRAF and one with PJA2/BRAF) in samples obtained at AR to EGFR TKI therapy (two posterlotinib ..
  2. Mayr J. Lipid metabolism in mitochondrial membranes. J Inherit Metab Dis. 2015;38:137-44 pubmed publisher
    ..Since a growing number of enzymes and transport processes are recognised as parts of the mitochondrial lipid metabolism, a further increase of lipid-related disorders can be expected. ..
  3. Yao L, Liu J, Xu L. MiR-610 functions as a tumor suppressor in oral squamous cell carcinoma by directly targeting AGK. Eur Rev Med Pharmacol Sci. 2019;23:187-197 pubmed publisher
    ..The regulation of acylglycerol kinase (AGK) by miR-610 was confirmed by Western blotting, Dual-Luciferase reporter assays and rescue experiments...
  4. Bhattacharjee P, De D, Bhattacharyya D. Degradation of fibrin-? amyloid co-aggregate: A novel function attributed to ubiquitin. Biochim Biophys Acta Mol Cell Res. 2018;1865:1465-1478 pubmed publisher
    ..These observations attribute a novel function of ubiquitin that may be used for degrading abnormal fibrin clots in human body. Thorough investigation might unfold a novel molecular mechanism of ubiquitin in protein homeostasis. ..
  5. Abu El Asrar A, Nawaz M, Mohammad G, Siddiquei M, Alam K, Mousa A, et al. Expression of bioactive lysophospholipids and processing enzymes in the vitreous from patients with proliferative diabetic retinopathy. Lipids Health Dis. 2014;13:187 pubmed publisher
    ..S1PR1 and SPL might regulate inflammatory, angiogenic and fibrogenic responses in PDR. ..
  6. Wortmann S, Espeel M, Almeida L, Reimer A, Bosboom D, Roels F, et al. Inborn errors of metabolism in the biosynthesis and remodelling of phospholipids. J Inherit Metab Dis. 2015;38:99-110 pubmed publisher
  7. Sun R, Weng H, Hu I, Guo J, Wu W, Zee B, et al. A W-test collapsing method for rare-variant association testing in exome sequencing data. Genet Epidemiol. 2016;40:591-596 pubmed publisher
    ..The proposed method offers an efficient and effective way for testing rare genetic variants in whole exome sequencing datasets. ..
  8. El Hattab A, Craigen W, Scaglia F. Mitochondrial DNA maintenance defects. Biochim Biophys Acta Mol Basis Dis. 2017;1863:1539-1555 pubmed publisher
  9. Jeong S, Cho G. The tubulin deacetylase sirtuin-2 regulates neuronal differentiation through the ERK/CREB signaling pathway. Biochem Biophys Res Commun. 2017;482:182-187 pubmed publisher
    ..Similar results were obtained for CREB phosphorylation (p-CREB). The results suggest that Sirt2 plays a crucial role in neuronal differentiation via the ERK-CREB signaling pathway. ..
  10. Cordioli M, Moraes L, Bastos A, Besson P, Alves M, Delcelo R, et al. Fusion Oncogenes Are the Main Genetic Events Found in Sporadic Papillary Thyroid Carcinomas from Children. Thyroid. 2017;27:182-188 pubmed publisher
    ..As mutations were unidentifiable in over 40% of the PTC cases, more comprehensive studies conducted in these patients will help to decipher the genetic landscape of sporadic pediatric PTCs. ..
  11. Toseef M, Saeed A, Mohi Ud Din E, Usmanghani K, Nazar H, Nawaz A, et al. Comparative clinical evaluation on herbal formulation Pepsil, Safoof-e-Katira and Omeprazole in gastro esophageal reflux disease. Pak J Pharm Sci. 2015;28:863-70 pubmed
    ..Matab Hakeem Muhammad Noor-ud-din, Burewala; Aziz Muhammad din Medical and Surgical Centre, Burewala and Shifa-ul-mulk Memorial Hospital, Hamdard University Karachi...
  12. Shimizu K, Quillinan N, Orfila J, Herson P. Sirtuin-2 mediates male specific neuronal injury following experimental cardiac arrest through activation of TRPM2 ion channels. Exp Neurol. 2016;275 Pt 1:78-83 pubmed publisher
    ..These results support the growing body of literature showing that oxidative stress mechanisms predominate in males and converge on TRPM2 activation as a mediator of cell death. ..
  13. Biella G, Fusco F, Nardo E, Bernocchi O, Colombo A, Lichtenthaler S, et al. Sirtuin 2 Inhibition Improves Cognitive Performance and Acts on Amyloid-? Protein Precursor Processing in Two Alzheimer's Disease Mouse Models. J Alzheimers Dis. 2016;53:1193-207 pubmed publisher
    ..Overall, our pre-clinical data support a role for SIRT2 inhibition in the improvement of cognitive performance and the modulation of molecular mechanisms relevant for AD, thus deserving attention as possible therapeutic strategy. ..
  14. Strickland K, Eszlinger M, Paschke R, Angell T, Alexander E, Marqusee E, et al. Molecular Testing of Nodules with a Suspicious or Malignant Cytologic Diagnosis in the Setting of Non-Invasive Follicular Thyroid Neoplasm with Papillary-Like Nuclear Features (NIFTP). Endocr Pathol. 2018;29:68-74 pubmed publisher
    ..However, molecular testing may be valuable for a subset of cases, especially those that are indeterminate for potential NIFTP/FVPTC versus cPTC based on cytologic features alone. ..
  15. Xu W, Liu L, Hornby D. c-IAP1 binds and processes PCSK9 protein: linking the c-IAP1 in a TNF-α pathway to PCSK9-mediated LDLR degradation pathway. Molecules. 2012;17:12086-101 pubmed publisher
    ..The novel pathway described here will open new avenues for exploring novel disease treatments. ..
  16. Spiegel S, Milstien S. Functions of the multifaceted family of sphingosine kinases and some close relatives. J Biol Chem. 2007;282:2125-9 pubmed
  17. Kang Y, Stroud D, Baker M, De Souza D, Frazier A, Liem M, et al. Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex. Mol Cell. 2017;67:457-470.e5 pubmed publisher
    b>Acylglycerol kinase (AGK) is a mitochondrial lipid kinase that catalyzes the phosphorylation of monoacylglycerol and diacylglycerol to lysophosphatidic acid and phosphatidic acid, respectively...
  18. Bastos A, de Jesus A, Cerutti J. ETV6-NTRK3 and STRN-ALK kinase fusions are recurrent events in papillary thyroid cancer of adult population. Eur J Endocrinol. 2018;178:83-91 pubmed publisher
    ..Further analysis is needed to define if they can also be useful for prognosis and guiding therapy. ..
  19. Mårtensson C, Becker T. Acylglycerol Kinase: Mitochondrial Protein Transport Meets Lipid Biosynthesis. Trends Cell Biol. 2017;27:700-702 pubmed publisher
    ..Recently, the acylglycerol kinase (AGK) mutated in Sengers syndrome was identified as a novel subunit of the human TIM22 complex...
  20. Abu El Asrar A, Mohammad G, Nawaz M, Siddiquei M, Kangave D, Opdenakker G. Expression of lysophosphatidic acid, autotaxin and acylglycerol kinase as biomarkers in diabetic retinopathy. Acta Diabetol. 2013;50:363-71 pubmed publisher
    ..This study was conducted to measure the levels of LPA and LPA-producing enzymes, autotaxin (ATX) and acylglycerol kinase (AGK) in the vitreous fluid from patients with proliferative diabetic retinopathy (PDR) and to correlate ..
  21. Allali S, Dorboz I, Samaan S, Slama A, Rambaud C, Boespflug Tanguy O, et al. Mutation in the AGK gene in two siblings with unusual Sengers syndrome. Metab Brain Dis. 2017;32:2149-2154 pubmed publisher
    Sengers syndrome is a rare autosomal recessive metabolic disorder caused by lack of acylglycerol kinase due to mutations in the AGK gene...
  22. Chi H. miR-194 regulated AGK and inhibited cell proliferation of oral squamous cell carcinoma by reducing PI3K-Akt-FoxO3a signaling. Biomed Pharmacother. 2015;71:53-7 pubmed publisher
    ..that miR-194 could reduce the phosphoinositide 3-kinase (PI3K)/AKT/FoxO3a signaling pathway by suppressing acylglycerol kinase (AGK) directly, resulting in decreasing cyclin D1 expression and increasing expression of p21 in OSCC...
  23. Liu N, Wang Z, Cheng Y, Zhang P, Wang X, Yang H, et al. Acylglycerol kinase functions as an oncogene and an unfavorable prognostic marker of human gliomas. Hum Pathol. 2016;58:105-112 pubmed publisher
    b>Acylglycerol kinase (AGK) regulates various cellular processes involved into tumorigenesis and tumor progression...
  24. Van Overloop H, Gijsbers S, Van Veldhoven P. Further characterization of mammalian ceramide kinase: substrate delivery and (stereo)specificity, tissue distribution, and subcellular localization studies. J Lipid Res. 2006;47:268-83 pubmed
    ..No evidence for phosphorylation of ceramide by the recently described multiple lipid kinase was found. The latter kinase is localized in the mitochondria, but no firm conclusions with regard to its substrate could be drawn. ..
  25. Vukotic M, Nolte H, König T, Saita S, Ananjew M, Kruger M, et al. Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria. Mol Cell. 2017;67:471-483.e7 pubmed publisher
    Mutations in mitochondrial acylglycerol kinase (AGK) cause Sengers syndrome, which is characterized by cataracts, hypertrophic cardiomyopathy, and skeletal myopathy...
  26. Haghighi A, Haack T, Atiq M, Mottaghi H, Haghighi Kakhki H, Bashir R, et al. Sengers syndrome: six novel AGK mutations in seven new families and review of the phenotypic and mutational spectrum of 29 patients. Orphanet J Rare Dis. 2014;9:119 pubmed publisher
    ..Mutations in the acylglycerol kinase (AGK) gene have been recently described as the cause of Sengers syndrome in nine families...
  27. Cui Y, Lin C, Wu Z, Liu A, Zhang X, Zhu J, et al. AGK enhances angiogenesis and inhibits apoptosis via activation of the NF-κB signaling pathway in hepatocellular carcinoma. Oncotarget. 2014;5:12057-69 pubmed
    ..Here, we demonstrated that acylglycerol kinase (AGK) is markedly overexpressed in HCC cell lines and clinical tissues...
  28. Zhai G, Teumer A, Stolk L, Perry J, Vandenput L, Coviello A, et al. Eight common genetic variants associated with serum DHEAS levels suggest a key role in ageing mechanisms. PLoS Genet. 2011;7:e1002025 pubmed publisher
    ..Several SNPs were associated with changes in gene expression levels, and the related genes are connected to biological pathways linking DHEAS with ageing. This study provides much needed insight into the function of DHEAS. ..
  29. Zhu Q, Cao S, Lin H, Yang Q, Liu S, Guo L. Overexpression of acylglycerol kinase is associated with poorer prognosis and lymph node metastasis in nasopharyngeal carcinoma. Tumour Biol. 2016;37:3349-57 pubmed publisher
    b>Acylglycerol kinase (AGK) has been reported to promote a malignant phenotype and enhance the development of cancer stem cells. However, the clinical value of AGK in cancer remains unclear...
  30. Calvo S, Compton A, Hershman S, Lim S, Lieber D, Tucker E, et al. Molecular diagnosis of infantile mitochondrial disease with targeted next-generation sequencing. Sci Transl Med. 2012;4:118ra10 pubmed publisher
    ..The pathogenicity of two such genes, NDUFB3 and AGK, was supported by complementation studies and evidence from multiple patients, respectively. The results underscore the potential and challenges of deploying NGS in clinical settings. ..
  31. Liu G, Ren X, Gao C, Zhang W. Acylglycerol kinase promotes the proliferation and cell cycle progression of oral squamous cell carcinoma. Mol Med Rep. 2015;12:2225-30 pubmed publisher
    ..b>Acylglycerol kinase (AGK) is a multisubstrate lipid kinase, which is known to be associated with the progression of various ..
  32. Sun F, Xiong Y, Zhou X, Li Q, Xiao L, Long P, et al. Acylglycerol kinase is over-expressed in early-stage cervical squamous cell cancer and predicts poor prognosis. Tumour Biol. 2016;37:6729-36 pubmed publisher
    b>Acylglycerol kinase (AGK) had been shown to contribute to cancer progression and unfavorable clinical outcomes of patients...