Dual specificity mitogen-activated protein kinase kinase 1
Also known as: MAP2K1, MEK1, PRKMK1
Function
Dual specificity protein kinase which acts as an essential component of the MAP kinase signal transduction pathway. Binding of extracellular ligands such as growth factors, cytokines and hormones to their cell-surface receptors activates RAS and this initiates RAF1 activation. RAF1 then further activates the dual-specificity protein kinases MAP2K1/MEK1 and MAP2K2/MEK2. Both MAP2K1/MEK1 and MAP2K2/MEK2 function specifically in the MAPK/ERK cascade, and catalyze the concomitant phosphorylation of a threonine and a tyrosine residue in a Thr-Glu-Tyr sequence located in the extracellular signal-regulated kinases MAPK3/ERK1 and MAPK1/ERK2, leading to their activation and further transduction of the signal within the MAPK/ERK cascade. Activates BRAF in a KSR1 or KSR2-dependent manner; by binding to KSR1 or KSR2 releases the inhibitory intramolecular interaction between KSR1 or KSR2 protein kinase and N-terminal domains which promotes KSR1 or KSR2-BRAF dimerization and BRAF activation. Depending on the cellular context, this pathway mediates diverse biological functions such as cell growth, adhesion, survival and differentiation, predominantly through the regulation of transcription, metabolism and cytoskeletal rearrangements. One target of the MAPK/ERK cascade is peroxisome proliferator-activated receptor gamma (PPARG), a nuclear receptor that promotes differentiation and apoptosis. MAP2K1/MEK1 has been shown to export PPARG from the nucleus. The MAPK/ERK cascade is also involved in the regulation of endosomal dynamics, including lysosome processing and endosome cycling through the perinuclear recycling compartment (PNRC), as well as in the fragmentation of the Golgi apparatus during mitosis.
Classification
- Family (Pfam)
- PF00069 Pkinase
- InterPro
- Kinase-like_dom_sf, MAP_kinase_kinase, Prot_kinase_dom, Protein_kinase_ATP_BS, Ser/Thr_kinase_AS
- Functional cluster
- Protein Serine/Threonine Kinases
Experimental structures · PDB · 87
- 1S9J X-ray 2.40A
- 2P55 X-ray 2.80A
- 3DV3 X-ray 2.30A
- 3DY7 X-ray 2.70A
- 3E8N X-ray 2.50A
- 3EQB X-ray 2.62A
- 3EQC X-ray 1.80A
- 3EQD X-ray 2.10A
- 3EQF X-ray 2.70A
- 3EQG X-ray 2.50A
- 3EQH X-ray 2.00A
- 3EQI X-ray 1.90A
- … and 75 more
A predicted model is available from AlphaFold.
Gene Ontology · 70
- GO:0030424 axon
- GO:0005938 cell cortex
- GO:0005813 centrosome
- GO:0036064 ciliary basal body
- GO:0005829 cytosol
- GO:0032839 dendrite cytoplasm
- GO:0005769 early endosome
- GO:0005783 endoplasmic reticulum
- GO:0005925 focal adhesion
- GO:0098978 glutamatergic synapse
- GO:0005794 Golgi apparatus
- GO:0005770 late endosome
- GO:0005874 microtubule
- GO:0005739 mitochondrion
- GO:0005634 nucleus
- GO:0043204 perikaryon
- GO:0048471 perinuclear region of cytoplasm
- GO:0005886 plasma membrane
- GO:0014069 postsynaptic density
- GO:0005524 ATP binding
- GO:0004708 MAP kinase kinase activity
- GO:0005078 MAP-kinase scaffold activity
- GO:0031435 mitogen-activated protein kinase kinase kinase binding
- GO:0030295 protein kinase activator activity
- GO:0004672 protein kinase activity
- GO:0106310 protein serine kinase activity
- GO:0043539 protein serine/threonine kinase activator activity
- GO:0004674 protein serine/threonine kinase activity
- GO:0004712 protein serine/threonine/tyrosine kinase activity
- GO:0004713 protein tyrosine kinase activity
- GO:0044877 protein-containing complex binding
- GO:0097110 scaffold protein binding
- GO:0031267 small GTPase binding
- GO:0090398 cellular senescence
- GO:0006935 chemotaxis
- GO:0038133 ERBB2-ERBB3 signaling pathway
- GO:0070371 ERK1 and ERK2 cascade
- GO:0048313 Golgi inheritance
- GO:0048009 insulin-like growth factor receptor signaling pathway
- GO:0000165 MAPK cascade
- GO:0032402 melanosome transport
- GO:0042552 myelination
- GO:0008285 negative regulation of cell population proliferation
- GO:0010629 negative regulation of gene expression
- GO:0034111 negative regulation of homotypic cell-cell adhesion
- GO:1903298 negative regulation of hypoxia-induced intrinsic apoptotic signaling pathway
- GO:0007528 neuromuscular junction development
- GO:0030182 neuron differentiation
- GO:0048812 neuron projection morphogenesis
- GO:2001171 positive regulation of ATP biosynthetic process
- GO:0010508 positive regulation of autophagy
- GO:0030335 positive regulation of cell migration
- GO:0045893 positive regulation of DNA-templated transcription
- GO:0070374 positive regulation of ERK1 and ERK2 cascade
- GO:0010628 positive regulation of gene expression
- GO:0045933 positive regulation of muscle contraction
- GO:0071902 positive regulation of protein serine/threonine kinase activity
- GO:0032968 positive regulation of transcription elongation by RNA polymerase II
- GO:2000641 regulation of early endosome to late endosome transport
- GO:0090170 regulation of Golgi inheritance
- GO:0098696 regulation of neurotransmitter receptor localization to postsynaptic specialization membrane
- GO:0032872 regulation of stress-activated MAPK cascade
- GO:0003056 regulation of vascular associated smooth muscle contraction
- GO:0048678 response to axon injury
- GO:0051384 response to glucocorticoid
- GO:0006979 response to oxidative stress
- GO:0014044 Schwann cell development
- GO:0007165 signal transduction
- GO:0070328 triglyceride homeostasis
- GO:0047496 vesicle transport along microtubule
Disease associations
- melorheostosis MONDO:0007970
- cardiofaciocutaneous syndrome 3 MONDO:0014113
Drugs targeting this protein · 16
- CI-1040 inhibitor
- E-6201 inhibitor
- REFAMETINIB inhibitor
- RO-4987655 inhibitor
- TAK-733 inhibitor
- TRAMETINIB inhibitor
- BALAMAPIMOD inhibitor
- SELUMETINIB SULFATE inhibitor
- TRAMETINIB DIMETHYL SULFOXIDE inhibitor
- PIMASERTIB inhibitor
- COBIMETINIB inhibitor
- COBIMETINIB FUMARATE inhibitor
- AZD-8330 inhibitor
- BINIMETINIB inhibitor
- AVUTOMETINIB inhibitor
- MIRDAMETINIB inhibitor
Related proteins · sequence + function similarity
- Dual specificity mitogen-activated protein kinase kinase 1 1.00
- Dual specificity mitogen-activated protein kinase kinase 1 1.00
- Dual specificity mitogen-activated protein kinase kinase 1 1.00
- Dual specificity mitogen-activated protein kinase kinase 1 1.00
- Dual specificity mitogen-activated protein kinase kinase 1 0.99
- Dual specificity mitogen-activated protein kinase kinase 1 0.99
- Dual specificity mitogen-activated protein kinase kinase 2 0.98
- Dual specificity mitogen-activated protein kinase kinase 1 0.98
- Dual specificity mitogen-activated protein kinase kinase 2 0.98
- Dual specificity mitogen-activated protein kinase kinase 2 0.98
- Dual specificity mitogen-activated protein kinase kinase 2 0.97
- Dual specificity mitogen-activated protein kinase kinase 2 0.96
Co-cited proteins · studied together in the literature
- Proto-oncogene serine/threonine-protein kinase mos 2 shared papers
- Dual specificity mitogen-activated protein kinase kinase 2 8 shared papers
- Serine/threonine-protein kinase STE7 1 shared papers
- Dual specificity mitogen-activated protein kinase kinase 1 6 shared papers
- Serine/threonine-protein kinase Nek10 1 shared papers
- Serine/threonine-protein kinase Nek10 1 shared papers
- Dual specificity mitogen-activated protein kinase kinase 1 4 shared papers
- TRAF3-interacting JNK-activating modulator 1 shared papers
- Kinase suppressor of Ras 1 3 shared papers
- Serine/threonine-protein kinase B-raf 3 shared papers
- TRAF3-interacting JNK-activating modulator 1 shared papers
- Dual specificity protein phosphatase 6 1 shared papers
Literature · 39 cited papers
- Biallelic variants in MOS cause large polar body in oocyte and human female infertility. Hum. Reprod. · 2022
- Biallelic mutations in MOS cause female infertility characterized by human early embryonic arrest and fragmentation. EMBO Mol. Med. · 2021
- Enhanced MAPK1 function causes a neurodevelopmental disorder within the RASopathy clinical spectrum. Am. J. Hum. Genet. · 2020
- Somatic activating mutations in MAP2K1 cause melorheostosis. Nat. Commun. · 2018
- MEK drives BRAF activation through allosteric control of KSR proteins. Nature · 2018
- T cell development involves TRAF3IP3-mediated ERK signaling in the Golgi. J. Exp. Med. · 2015
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- The ERK cascade: distinct functions within various subcellular organelles. Genes Cancer · 2011
- Design and synthesis of novel allosteric MEK inhibitor CH4987655 as an orally available anticancer agent. Bioorg. Med. Chem. Lett. · 2011
- Discovery of TAK-733, a potent and selective MEK allosteric site inhibitor for the treatment of cancer. Bioorg. Med. Chem. Lett. · 2011
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Nek10 mediates G2/M cell cycle arrest and MEK autoactivation in response to UV irradiation. Mol. Cell. Biol. · 2011
- … and 27 more in the literature graph