NF2 - Wiki-MPM

Interactant Symbol	Name	Associated Pathways Hide	Binding Drugs Hide	Associated Diseases Hide
FGFR4	fibroblast growth factor receptor 4	PI3K Cascade PIP3 activates AKT signaling betaKlotho-mediated ligand binding FGFR4 mutant receptor activation FGFR4 ligand binding and activation Constitutive Signaling by Aberrant PI3K in Cancer Phospholipase C-mediated cascade; FGFR4 FRS-mediated FGFR4 signaling SHC-mediated cascade:FGFR4 PI-3K cascade:FGFR4 Negative regulation of FGFR4 signaling Signaling by FGFR4 in disease RAF/MAP kinase cascade PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling	Heparin Ponatinib Lenvatinib Erdafitinib Pemigatinib
XPO1	exportin 1	Amplification of signal from unattached kinetochores via a MAD2 inhibitory signal Rev-mediated nuclear export of HIV RNA NEP/NS2 Interacts with the Cellular Export Machinery Downregulation of TGF-beta receptor signaling Separation of Sister Chromatids Resolution of Sister Chromatid Cohesion Deactivation of the beta-catenin transactivating complex HuR (ELAVL1) binds and stabilizes mRNA RHO GTPases Activate Formins MAPK6/MAPK4 signaling Mitotic Prometaphase Cyclin A/B1/B2 associated events during G2/M transition Estrogen-dependent nuclear events downstream of ESR-membrane signaling EML4 and NUDC in mitotic spindle formation	Selinexor
PDGFRA	platelet derived growth factor receptor alpha	PIP3 activates AKT signaling Downstream signal transduction Signaling by PDGF Constitutive Signaling by Aberrant PI3K in Cancer RAF/MAP kinase cascade PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling Signaling by PDGFRA transmembrane, juxtamembrane and kinase domain mutants Signaling by PDGFRA extracellular domain mutants Imatinib-resistant PDGFR mutants Sunitinib-resistant PDGFR mutants Regorafenib-resistant PDGFR mutants Sorafenib-resistant PDGFR mutants PDGFR mutants bind TKIs	Becaplermin Imatinib Sunitinib XL820 Olaratumab Pazopanib Midostaurin Regorafenib Ponatinib Lenvatinib Nintedanib Foreskin keratinocyte (neonatal) Fostamatinib Erdafitinib Amuvatinib Ripretinib	Glioma
FZR1	fizzy and cell division cycle 20 related 1	Autodegradation of Cdh1 by Cdh1:APC/C SCF-beta-TrCP mediated degradation of Emi1 APC/C:Cdh1 mediated degradation of Cdc20 and other APC/C:Cdh1 targeted proteins in late mitosis/early G1 Conversion from APC/C:Cdc20 to APC/C:Cdh1 in late anaphase Regulation of APC/C activators between G1/S and early anaphase Phosphorylation of Emi1 Senescence-Associated Secretory Phenotype (SASP) CDK-mediated phosphorylation and removal of Cdc6 Cyclin A:Cdk2-associated events at S phase entry Transcriptional Regulation by VENTX Aberrant regulation of mitotic exit in cancer due to RB1 defects Antigen processing: Ubiquitination & Proteasome degradation
HGS	hepatocyte growth factor-regulated tyrosine kinase substrate	EGFR downregulation Lysosome Vesicle Biogenesis Ub-specific processing proteases Negative regulation of MET activity Cargo recognition for clathrin-mediated endocytosis Clathrin-mediated endocytosis InlB-mediated entry of Listeria monocytogenes into host cell Endosomal Sorting Complex Required For Transport (ESCRT) Inhibition of membrane repair Prevention of phagosomal-lysosomal fusion RHOBTB3 ATPase cycle	Citric acid
ARAF	A-Raf proto-oncogene, serine/threonine kinase	RAF activation MAP2K and MAPK activation Negative regulation of MAPK pathway Signaling by moderate kinase activity BRAF mutants Signaling by high-kinase activity BRAF mutants Signaling by BRAF and RAF fusions Paradoxical activation of RAF signaling by kinase inactive BRAF Signaling downstream of RAS mutants Signaling by RAF1 mutants Signaling by MRAS-complex mutants	ATP	Noonan syndrome and related disorders, including: Noonan syndrome (NS); Leopard syndrome (LS); Noonan syndrome-like with loose anagen hair (NS/LAH); CBL-mutation associated syndrome (CBL); Neurofibromatosis type 1 (NF1); Neurofibromatosis type 2 (NF2); Neurofibromatosis-Noonan syndrome (NFNS); Legius syndrome; Cardiofaciocutaneous syndrome (CFCS); Costello syndrome (CS)
HIF1A	hypoxia inducible factor 1 subunit alpha	Regulation of gene expression by Hypoxia-inducible Factor Cellular response to hypoxia Oxygen-dependent proline hydroxylation of Hypoxia-inducible Factor Alpha NOTCH1 Intracellular Domain Regulates Transcription Circadian Clock Ub-specific processing proteases Interleukin-4 and Interleukin-13 signaling PTK6 Expression PTK6 promotes HIF1A stabilization Neddylation	Carvedilol Hydralazine 2-Methoxyestradiol ENMD-1198 PX-478 FG-2216
SPTBN1	spectrin beta, non-erythrocytic 1	Nephrin family interactions NCAM signaling for neurite out-growth NCAM signaling for neurite out-growth Interaction between L1 and Ankyrins Interaction between L1 and Ankyrins RAF/MAP kinase cascade COPI-mediated anterograde transport Signaling by FLT3 fusion proteins Signaling by FLT3 fusion proteins	Calcium 1D-myo-inositol 1,4,5-trisphosphate
AURKA	aurora kinase A	APC/C:Cdh1 mediated degradation of Cdc20 and other APC/C:Cdh1 targeted proteins in late mitosis/early G1 Regulation of PLK1 Activity at G2/M Transition SUMOylation of DNA replication proteins TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest Regulation of TP53 Activity through Phosphorylation FBXL7 down-regulates AURKA during mitotic entry and in early mitosis AURKA Activation by TPX2 Interaction between PHLDA1 and AURKA	Phosphonothreonine AT9283 CYC116 Alisertib SNS-314 Cenisertib Enzastaurin 4-(4-METHYLPIPERAZIN-1-YL)-N-[5-(2-THIENYLACETYL)-1,5-DIHYDROPYRROLO[3,4-C]PYRAZOL-3-YL]BENZAMIDE AKI-001 1-{5-[2-(thieno[3,2-d]pyrimidin-4-ylamino)ethyl]-1,3-thiazol-2-yl}-3-[3-(trifluoromethyl)phenyl]urea 1-(5-{2-[(1-methyl-1H-pyrazolo[4,3-d]pyrimidin-7-yl)amino]ethyl}-1,3-thiazol-2-yl)-3-[3-(trifluoromethyl)phenyl]urea N-{3-[(4-{[3-(TRIFLUOROMETHYL)PHENYL]AMINO}PYRIMIDIN-2-YL)AMINO]PHENYL}CYCLOPROPANECARBOXAMIDE N-butyl-3-{[6-(9H-purin-6-ylamino)hexanoyl]amino}benzamide 2-(1H-pyrazol-3-yl)-1H-benzimidazole N-[3-(1H-BENZIMIDAZOL-2-YL)-1H-PYRAZOL-4-YL]BENZAMIDE Fostamatinib MLN8054
CDK6	cyclin dependent kinase 6	Oxidative Stress Induced Senescence Senescence-Associated Secretory Phenotype (SASP) Oncogene Induced Senescence Cyclin D associated events in G1 Regulation of RUNX1 Expression and Activity Evasion of Oncogene Induced Senescence Due to Defective p16INK4A binding to CDK4 and CDK6 Evasion of Oxidative Stress Induced Senescence Due to Defective p16INK4A binding to CDK4 and CDK6 Defective binding of RB1 mutants to E2F1,(E2F2, E2F3)	Alvocidib (2S)-2-({6-[(3-Amino-5-chlorophenyl)amino]-9-isopropyl-9H-purin-2-yl}amino)-3-methyl-1-butanol Fisetin Palbociclib Ribociclib Abemaciclib
KDM1A	lysine demethylase 1A	HDACs deacetylate histones HDMs demethylate histones Activated PKN1 stimulates transcription of AR (androgen receptor) regulated genes KLK2 and KLK3 Regulation of PTEN gene transcription Estrogen-dependent gene expression NR1H3 & NR1H2 regulate gene expression linked to cholesterol transport and efflux Potential therapeutics for SARS Factors involved in megakaryocyte development and platelet production
MDM4	MDM4 regulator of p53	Oxidative Stress Induced Senescence Oncogene Induced Senescence Ub-specific processing proteases Regulation of TP53 Activity through Phosphorylation Regulation of TP53 Degradation Regulation of TP53 Activity through Methylation Stabilization of p53
ARNT	aryl hydrocarbon receptor nuclear translocator	Regulation of gene expression by Hypoxia-inducible Factor PPARA activates gene expression Phase I - Functionalization of compounds Endogenous sterols Xenobiotics Aryl hydrocarbon receptor signalling
CD44	CD44 molecule (Indian blood group)	Degradation of the extracellular matrix Cell surface interactions at the vascular wall Integrin cell surface interactions Hyaluronan uptake and degradation Neutrophil degranulation Interferon gamma signaling	Bivatuzumab Hyaluronic acid	Gastric cancer
EIF3B	eukaryotic translation initiation factor 3 subunit B	L13a-mediated translational silencing of Ceruloplasmin expression Translation initiation complex formation Formation of a pool of free 40S subunits Formation of the ternary complex, and subsequently, the 43S complex Ribosomal scanning and start codon recognition GTP hydrolysis and joining of the 60S ribosomal subunit
EPHA2	EPH receptor A2	EPH-Ephrin signaling EPH-Ephrin signaling EPHA-mediated growth cone collapse EPHA-mediated growth cone collapse EPH-ephrin mediated repulsion of cells EPH-ephrin mediated repulsion of cells	Dasatinib Phosphoaminophosphonic Acid-Adenylate Ester Regorafenib Fostamatinib
PXN	paxillin	GAB1 signalosome VEGFA-VEGFR2 Pathway Smooth Muscle Contraction Localization of the PINCH-ILK-PARVIN complex to focal adhesions Regulation of cytoskeletal remodeling and cell spreading by IPP complex components PTK6 Regulates RHO GTPases, RAS GTPase and MAP kinases
CDKN2B	cyclin dependent kinase inhibitor 2B	SMAD2/SMAD3:SMAD4 heterotrimer regulates transcription Oxidative Stress Induced Senescence Senescence-Associated Secretory Phenotype (SASP) Oncogene Induced Senescence Cyclin D associated events in G1		Malignant pleural mesothelioma Osteosarcoma Type II diabetes mellitus
EMD	emerin	Nuclear Envelope Breakdown Initiation of Nuclear Envelope (NE) Reformation Depolymerisation of the Nuclear Lamina Insertion of tail-anchored proteins into the endoplasmic reticulum membrane Insertion of tail-anchored proteins into the endoplasmic reticulum membrane		Emery-Dreifuss muscular dystrophy
SDCBP	syndecan binding protein	Ephrin signaling Neurofascin interactions RIPK1-mediated regulated necrosis Regulation of necroptotic cell death Neutrophil degranulation

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