NF2

Summary

NF2 (NF2, moesin-ezrin-radixin like (MERLIN) tumor suppressor, HGNC:7773) is a protein-coding gene on chromosome 22q12.2, encoding Merlin (P35240). Probable regulator of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway, a signaling pathway that plays a pivotal role in tumor suppression by restricting proliferation and promoting apoptosis. In precision oncology, NF2 K159fs confers sensitivity to Temsirolimus in Breast Cancer (CIViC Level C); 2 further curated variant–drug associations are listed below. It is haploinsufficient (ClinGen: sufficient evidence).

This gene encodes a protein that is similar to some members of the ERM (ezrin, radixin, moesin) family of proteins that link cytoskeletal components with proteins in the cell membrane. The encoded protein is involved in regulation of contact-dependent inhibition of cell proliferation and functions in cell-cell adhesion and transmembrane signaling. The encoded protein has been shown to interact with cell-surface proteins, proteins involved in cytoskeletal dynamics, and proteins involved in regulating ion transport. Disruption of this protein’s function has been implicated in tumorigenesis and metastasis. Mutations in this gene are associated with neurofibromatosis type II which is characterized by nervous system and skin tumors and ocular abnormalities.

Source: NCBI Gene 4771 — RefSeq curated summary.

At a glance

• Gene–disease (curated): NF2-related schwannomatosis (Definitive, ClinGen) — +1 more curated relationship
• GWAS associations: 1
• Clinical variants (ClinVar): 2,555 total — 260 pathogenic, 51 likely-pathogenic
• Phenotypes (HPO): 137
• Precision-oncology evidence (CIViC): 3 curated variant–drug associations
• Cancer driver (intOGen): loss-of-function (tumor-suppressor-like) across 10 cancer types
• Dosage sensitivity (ClinGen): haploinsufficiency sufficient evidence, triplosensitivity no evidence
• MANE Select transcript: NM_000268

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:7773
Approved symbol	NF2
Name	NF2, moesin-ezrin-radixin like (MERLIN) tumor suppressor
Location	22q12.2
Locus type	gene with protein product
Status	Approved
Aliases	merlin, ACN, SCH, BANF, merlin-1
Ensembl gene	ENSG00000186575
Ensembl biotype	protein_coding
OMIM	607379
Entrez	4771

Gene structure

Transcript identifiers

Ensembl transcripts: 21 — 17 protein_coding, 4 nonsense_mediated_decay

ENST00000334961, ENST00000338641, ENST00000353887, ENST00000361166, ENST00000361452, ENST00000361676, ENST00000397789, ENST00000403435, ENST00000403999, ENST00000413209, ENST00000432151, ENST00000672461, ENST00000672805, ENST00000672896, ENST00000673312, ENST00000713935, ENST00000906700, ENST00000906701, ENST00000906702, ENST00000912971, ENST00000958075

RefSeq mRNA: 23 — MANE Select: NM_000268 NM_000268, NM_001407053, NM_001407054, NM_001407055, NM_001407056, NM_001407057, NM_001407058, NM_001407059, NM_001407060, NM_001407062, NM_001407063, NM_001407064, NM_001407065, NM_001407066, NM_001407067, NM_016418, NM_181825, NM_181828, NM_181829, NM_181830, NM_181831, NM_181832, NM_181833

CCDS: CCDS13861, CCDS13862, CCDS13863, CCDS13864, CCDS13865, CCDS54516

Canonical transcript exons

ENST00000338641 — 16 exons

Exon	Start	End
ENSE00000652099	29642202	29642285
ENSE00001171309	29639090	29639212
ENSE00001292557	29655594	29655676
ENSE00001305681	29658189	29658264
ENSE00001306896	29664990	29665064
ENSE00001323333	29654657	29654725
ENSE00001325971	29668333	29668446
ENSE00001327284	29673269	29673486
ENSE00001373499	29636751	29636876
ENSE00001383963	29661205	29661339
ENSE00001557625	29671826	29671948
ENSE00001641610	29694752	29698598
ENSE00003551311	29674836	29674941
ENSE00003570003	29681439	29681601
ENSE00003641081	29678196	29678323
ENSE00003904031	29603633	29604112

Expression profiles

Bgee: expression breadth ubiquitous, 283 present calls, max score 91.56.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 48.4823 / max 359.8614, expressed in 1820 samples.

FANTOM5 promoters (6 alternative TSS)

Promoter ID	TPM avg	Samples expressed
191623	44.2951	1819
191624	2.2694	1186
209445	0.7389	511
209446	0.5393	329
191627	0.3531	76
191629	0.2866	103

Top tissues by expression

293 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
endometrium epithelium	UBERON:0004811	91.56	gold quality
stromal cell of endometrium	CL:0002255	91.46	gold quality
dorsal motor nucleus of vagus nerve	UBERON:0002870	90.99	gold quality
tendon of biceps brachii	UBERON:0008188	90.78	gold quality
CA1 field of hippocampus	UBERON:0003881	90.19	gold quality
gluteal muscle	UBERON:0002000	90.00	gold quality
postcentral gyrus	UBERON:0002581	89.07	gold quality
vastus lateralis	UBERON:0001379	88.62	gold quality
tendon	UBERON:0000043	88.48	gold quality
parietal lobe	UBERON:0001872	88.01	gold quality
triceps brachii	UBERON:0001509	87.91	silver quality
entorhinal cortex	UBERON:0002728	87.86	gold quality
inferior olivary complex	UBERON:0002127	87.65	gold quality
calcaneal tendon	UBERON:0003701	87.65	gold quality
Ammon’s horn	UBERON:0001954	87.53	gold quality
quadriceps femoris	UBERON:0001377	87.51	gold quality
substantia nigra pars compacta	UBERON:0001965	87.18	gold quality
adrenal tissue	UBERON:0018303	87.14	gold quality
Brodmann (1909) area 46	UBERON:0006483	87.12	gold quality
skeletal muscle tissue	UBERON:0001134	86.98	gold quality
cingulate cortex	UBERON:0003027	86.94	gold quality
skeletal muscle tissue of biceps brachii	UBERON:0004502	86.90	gold quality
gingival epithelium	UBERON:0001949	86.87	gold quality
anterior cingulate cortex	UBERON:0009835	86.81	gold quality
gingiva	UBERON:0001828	86.77	gold quality
superior frontal gyrus	UBERON:0002661	86.77	gold quality
right frontal lobe	UBERON:0002810	86.66	gold quality
amygdala	UBERON:0001876	86.58	gold quality
temporal lobe	UBERON:0001871	86.55	gold quality
muscle organ	UBERON:0001630	86.48	gold quality

Single-cell (SCXA)

Detected in 2 experiment(s), a significant marker in 1.

Experiment	Marker?	Max mean expression
E-ANND-3	yes	8.75
E-GEOD-124858	no	316.64

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): AR, SP1

miRNA regulators (miRDB)

154 targeting NF2, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-3613-3P	100.00	76.36	7965
HSA-MIR-3689D	100.00	66.14	1181
HSA-MIR-6851-5P	100.00	65.63	1294
HSA-MIR-8485	100.00	77.57	4731
HSA-MIR-6758-5P	100.00	66.21	1470
HSA-MIR-6856-5P	100.00	65.47	1298
HSA-MIR-4481	100.00	66.42	1669
HSA-MIR-371B-5P	99.99	75.34	4759
HSA-MIR-4531	99.99	69.70	3181
HSA-MIR-548C-3P	99.99	74.01	7587
HSA-MIR-373-5P	99.98	75.36	4753
HSA-MIR-616-5P	99.98	75.58	4775
HSA-MIR-25-3P	99.98	74.60	1817
HSA-MIR-32-5P	99.98	75.21	1964
HSA-MIR-363-3P	99.98	74.72	1821
HSA-MIR-367-3P	99.98	74.83	1819
HSA-MIR-92A-3P	99.98	75.21	1960
HSA-MIR-92B-3P	99.98	75.25	1955
HSA-MIR-5696	99.98	72.36	4487
HSA-MIR-4725-3P	99.96	69.53	2520
HSA-MIR-6780B-5P	99.96	69.60	2562
HSA-MIR-4267	99.96	66.53	2368
HSA-MIR-4487	99.96	64.58	1252
HSA-MIR-497-5P	99.92	71.83	2674
HSA-MIR-15B-5P	99.90	72.78	2798
HSA-MIR-4731-5P	99.89	67.23	2537
HSA-MIR-7162-3P	99.89	68.16	1682
HSA-MIR-6780A-5P	99.88	66.69	2776
HSA-MIR-4271	99.88	68.32	2244
HSA-MIR-4492	99.87	68.25	3611

Functional genomics

ClinGen dosage: haploinsufficiency 3 (sufficient evidence), triplosensitivity 0 (no evidence). ClinGen Gene Dosage Map

Literature-anchored findings (GeneRIF, showing 40)

• A constitutional de novo interstitial deletion of 8 Mb on chromosome 22q12.1-12.3 encompassing the neurofibromatosis type 2 (NF2) locus in a dysmorphic girl with severe malformations. (PMID:11836375)
• Nonsense NF2 mutations and NF2 allele losses were found in all tumors in children of sporadic neurofibromatosis 2 patients in whom no NF2-mutations were found by screening their blood-DNA. (PMID:11839955)
• The motor protein kinesin-1 links neurofibromin and this protein in a common cellular pathway of neurofibromatosis (PMID:12191989)
• The binding of merlin to its partner, hepatocyte growth factor-regulated tyrosine kinase substrate, may facilitate its ability to function as a tumor suppressor. (PMID:12444101)
• Schwannomin inhibits Stat3 activation in schwannoma cells. (PMID:12444102)
• isolation and characterization of an aggresome determinant in this tumor suppressor gene (PMID:12471027)
• NF2 has a role in the development of multiple meningioma (PMID:12478663)
• Small NF2 mutations were identified in six out of seventeen meningiomas and schwannomas. Large deletions occurred in six meningiomas. These mutations could cause a truncated NF2 protein or loss of a large protein domain. (PMID:12665675)
• functional loss of the NF2 protein may be involved in the formation of a subset of human malignant mesothelioma (PMID:12684666)
• merlin is the first neuronal binding partner for PKA-RIbeta and may have a novel function in connecting neuronal cytoskeleton to PKA signaling (PMID:12896975)
• The lack of NF2 alterations supports the hypothesis that GI schwannomas represent a morphologically and genetically distinct group of peripheral nerve sheath tumors that are different from conventional schwannomas. (PMID:13679444)
• Review discusses NF2 involvement in cell motility, cell proliferation, and Rac signaling, and its implication in the development of sporadic schwannomas and meningiomas. (PMID:14566860)
• NF2 has a role in inhibiting schwannoma cell proliferation through promoting PDGFR degradation (PMID:14612918)
• the first direct demonstration that the S518D merlin mutation, which mimics merlin phosphorylation, impairs not only merlin growth and motility suppression but also leads to a novel phenotype previously ascribed to ERM proteins (PMID:14724586)
• both the merlin N and C termini are phosphorylated by PKA; phosphorylation of serine 518 promotes heterodimerization between merlin and ezrin (PMID:14981079)
• interacts with transactivation-responsive RNA-binding protein and inhibits its oncogenic activity; results provide the first clue to functional interaction between TRBP and merlin and suggest a novel mechanism for the tumor suppressor function of merlin (PMID:15123692)
• S518 phosphorylation modulates ability of merlin to function as a tumor suppressor (PMID:15378014)
• role for merlin in receptor-mediated signaling at the cell surface (PMID:15467741)
• In glioma and osteosarcoma cells, endogenous merlin was targeted to the nucleus in a cell cycle-specific manner. (PMID:15580288)
• Aberrant NF2 hypermethylation is associated with the development of meningiomas (PMID:15609345)
• Mosaic NF2 may be the cause of about 8% of multiple meningiomas in sporadic adult cases. (PMID:15635074)
• NF2 tumor suppressor Merlin and the ERM proteins interact with N-WASP and regulate its actin polymerization function (PMID:15699051)
• Neurofibromin 2 (merlin) exerts antiproliferative effect via repression of PAK-induced cyclin D1 expression. (PMID:15743831)
• The widespread expression of merlin in brain and its association with protein kinase A suggest a role for merlin in brain biology. (PMID:15797715)
• Involvement of the neurofibromatosis 2 tumour suppressor gene, NF2, on chromosome 22q in the high incidence of benign meningioma in the elderly. (PMID:15980114)
• Merlin can function as a tumor suppressor by inhibiting the RalGDS-mediated oncogenic signals. (PMID:16007223)
• Schwannomin inhibits tumorigenesis through direct interaction with the eukaryotic initiation factor subunit c (eIF3c) (PMID:16497727)
• Localization of HEI10 is dependent on merlin expression level. (PMID:16532029)
• Merlin and MLK3 can interact in situ and merlin can disrupt the interactions between B-Raf and Raf-1 or those between MLK3 and either B-Raf or Raf-1. (PMID:16537381)
• transitional and fibroblastic meningiomas harbor significantly more NF2 mutations than meningothelial meningiomas, indicating molecular subsets of these tumors (PMID:16612978)
• Merlin negatively regulates focal adhesion kinase, a major component of pathways that control cell motility and invasiveness. (PMID:16652148)
• Mutational analysis by denaturing HPLC showed that mutations in the NF2 gene play an important role in the development of sporadic meningiomas. (PMID:16786152)
• Functional duality of merlin may represent a paradigm in proteome complexity and is important in investigating multifactorial diseases such as cancer. (PMID:16824698)
• CPI-17 siRNA decreased the level of merlin phosphorylation and consequently Ras and ERK activity in human tumor cell lines (PMID:16885985)
• Merlin was expressed in vestibular schwannoma tissue. There were no correlations between merlin expression percentage and the age, gender, tumor diameter and clinical stage. (PMID:17007372)
• NF2-associated GTP binding protein is a tumor suppressor that regulates and requires merlin to suppress cell proliferation (PMID:17210637)
• There is a higher frequency of biallelic NF2 inactivation in fibroblastic (52%) versus meningothelial (18%) tumors, presence of macro-mutations on 22q also shows marked differences between fibroblastic (86%) and meningothelial (39%) meningioma subtypes. (PMID:17222329)
• The NF2 gene plays a role in the tumorigenesis of pediatric meningiomas and chromogenic in situ hybridization detects NF2 gene deletion in formalin-fixed, paraffin-embedded tissues. (PMID:17478763)
• Schwannomas and meningiomas, and to a lesser degree, ependymomas, express a high incidence of NF2 gene deletion, which supports the hypothesis that NF2 gene plays an important role in their tumorigenesis. (PMID:17509660)
• merlin plays a key role in the regulation of the Schwann cell microtubule cytoskeleton (PMID:17566081)

Cross-species orthologs

5 orthologs

Organism	Symbol	Gene ID
danio_rerio	nf2a	ENSDARG00000020204
mus_musculus	Nf2	ENSMUSG00000009073
rattus_norvegicus	Nf2	ENSRNOG00000007948
drosophila_melanogaster	Mer	FBGN0086384
caenorhabditis_elegans		WBGENE00003593

Paralogs (6): EZR (ENSG00000092820), FRMD4B (ENSG00000114541), ERMN (ENSG00000136541), RDX (ENSG00000137710), MSN (ENSG00000147065), FRMD4A (ENSG00000151474)

Protein

Protein identifiers

Merlin — P35240 (reviewed: P35240)

Alternative names: Moesin-ezrin-radixin-like protein, Neurofibromin-2, Schwannomerlin, Schwannomin

All UniProt accessions (4): A0A5F9ZHA0, A0A5F9ZHD8, A0A5K1VW66, P35240

UniProt curated annotations — full annotation on UniProt →

Function. Probable regulator of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway, a signaling pathway that plays a pivotal role in tumor suppression by restricting proliferation and promoting apoptosis. Along with WWC1 can synergistically induce the phosphorylation of LATS1 and LATS2 and can probably function in the regulation of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway. May act as a membrane stabilizing protein. May inhibit PI3 kinase by binding to AGAP2 and impairing its stimulating activity. Suppresses cell proliferation and tumorigenesis by inhibiting the CUL4A-RBX1-DDB1-VprBP/DCAF1 E3 ubiquitin-protein ligase complex.

Subunit / interactions. Interacts with NHERF1, HGS and AGAP2. Interacts with LAYN. Interacts with SGSM3. Interacts (via FERM domain) with MPP1. Interacts with WWC1. Interacts with the CUL4A-RBX1-DDB1-VprBP/DCAF1 E3 ubiquitin-protein ligase complex. The unphosphorylated form interacts (via FERM domain) with VPRBP/DCAF1. Interacts (via FERM domain) with NOP53; the interaction is direct. Interacts with SCHIP1; the interaction is direct.

Subcellular location. Cell projection. Filopodium membrane. Ruffle membrane. Nucleus Cytoplasm. Perinuclear region. Cytoplasmic granule Cytoplasm. Cytoplasmic granule Nucleus. Cytoplasm. Cytoplasmic granule. Cytoskeleton.

Tissue specificity. Widely expressed. Isoform 1 and isoform 3 are predominant. Isoform 4, isoform 5 and isoform 6 are expressed moderately. Isoform 8 is found at low frequency. Isoform 7, isoform 9 and isoform 10 are not expressed in adult tissues, with the exception of adult retina expressing isoform 10. Isoform 9 is faintly expressed in fetal brain, heart, lung, skeletal muscle and spleen. Fetal thymus expresses isoforms 1, 7, 9 and 10 at similar levels.

Post-translational modifications. Phosphorylation of Ser-518 inhibits nuclear localization by disrupting the intramolecular association of the FERM domain with the C-terminal tail. The dephosphorylation of Ser-518 favors the interaction with NOP53. Ubiquitinated by the CUL4A-RBX1-DDB1-DCAF1/VprBP E3 ubiquitin-protein ligase complex for ubiquitination and subsequent proteasome-dependent degradation.

Disease relevance. Schwannomatosis, vestibular (SWNV) [MIM:101000] An autosomal dominant neoplasia syndrome characterized by the development of multiple benign nerve sheath tumors called schwannomas, particularly affecting the vestibular nerve. Affected individuals usually present with bilateral vestibular schwannomas but can have schwannomas on other cranial, spinal, and peripheral/cutaneous nerves. Meningiomas are common, whereas 20 to 35% of affected individuals develop intramedullary spinal cord tumors called ependymomas. The condition is also characterized by several ophthalmic features such as lenticular opacities, retinal hamartoma, epiretinal membranes. The disease is caused by variants affecting the gene represented in this entry. Mesothelioma, malignant (MESOM) [MIM:156240] An aggressive neoplasm of the serosal lining of the chest. It appears as broad sheets of cells, with some regions containing spindle-shaped, sarcoma-like cells and other regions showing adenomatous patterns. Pleural mesotheliomas have been linked to exposure to asbestos. The disease may be caused by variants affecting the gene represented in this entry.

Isoforms (10)

UniProt ID	Names	Canonical?
P35240-1	1, I	yes
P35240-2	2
P35240-3	3, II
P35240-4	4, delE2/3
P35240-5	5, delE3
P35240-6	6, delE2
P35240-7	7, MER150
P35240-8	8
P35240-9	9, MER162
P35240-10	10, MER151

RefSeq proteins (23): NP_000259, NP_001393982, NP_001393983, NP_001393984, NP_001393985, NP_001393986, NP_001393987, NP_001393988, NP_001393989, NP_001393991, NP_001393992, NP_001393993, NP_001393994, NP_001393995, NP_001393996, NP_057502, NP_861546, NP_861966, NP_861967, NP_861968, NP_861969, NP_861970, NP_861971 (=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR000299	FERM_domain	Domain
IPR000798	Ez/rad/moesin-like	Family
IPR008954	Moesin_tail_sf	Homologous_superfamily
IPR011174	ERM	Family
IPR011259	ERM_C_dom	Domain
IPR011993	PH-like_dom_sf	Homologous_superfamily
IPR014352	FERM/acyl-CoA-bd_prot_sf	Homologous_superfamily
IPR018979	FERM_N	Domain
IPR018980	FERM_PH-like_C	Domain
IPR019747	FERM_CS	Conserved_site
IPR019748	FERM_central	Domain
IPR019749	Band_41_domain	Domain
IPR029071	Ubiquitin-like_domsf	Homologous_superfamily
IPR035963	FERM_2	Homologous_superfamily
IPR041789	ERM_FERM_C	Domain
IPR046810	ERM_helical	Domain

Pfam: PF00373, PF00769, PF09379, PF09380, PF20492

UniProt features (87 total): sequence variant 29, helix 16, strand 15, splice variant 13, turn 5, mutagenesis site 3, modified residue 2, sequence conflict 2, chain 1, domain 1

Structure

Experimental structures (PDB)

6 structures.

PDB	Method	Resolution (Å)
7LWH	X-RAY DIFFRACTION	1.61
1H4R	X-RAY DIFFRACTION	1.8
4ZRJ	X-RAY DIFFRACTION	2.3
6CDS	X-RAY DIFFRACTION	2.62
3U8Z	X-RAY DIFFRACTION	2.64
4ZRI	X-RAY DIFFRACTION	2.7

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-P35240-F1	87.85	0.67

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Post-translational modifications (2): 13, 518

Mutagenesis-validated functional residues (3):

Position	Phenotype
64	abolishes binding to agap2 and interaction with the cul4a-rbx1-ddb1-vprbp/dcaf1 e3 ubiquitin-protein ligase complex.
518	loss of phosphorylation. significant accumulation in the nucleus and no effect on binding to dcaf1.
518	no effect on phosphorylation. defective nuclear accumulation. significant decrease in binding to dcaf1 and in ability to

Function

Pathways and Gene Ontology

Reactome pathways

9 pathways

ID	Pathway
R-HSA-2029482	Regulation of actin dynamics for phagocytic cup formation
R-HSA-5627123	RHO GTPases activate PAKs
R-HSA-162582	Signal Transduction
R-HSA-168249	Innate Immune System
R-HSA-168256	Immune System
R-HSA-194315	Signaling by Rho GTPases
R-HSA-195258	RHO GTPase Effectors
R-HSA-2029480	Fcgamma receptor (FCGR) dependent phagocytosis
R-HSA-9716542	Signaling by Rho GTPases, Miro GTPases and RHOBTB3

MSigDB gene sets: 641 (showing top): GOBP_LENS_FIBER_CELL_DIFFERENTIATION, GOBP_EPITHELIUM_DEVELOPMENT, GOBP_ACTIN_FILAMENT_BUNDLE_ORGANIZATION, BUYTAERT_PHOTODYNAMIC_THERAPY_STRESS_DN, REACTOME_INNATE_IMMUNE_SYSTEM, GRUETZMANN_PANCREATIC_CANCER_DN, GOBP_REGULATION_OF_CELL_MORPHOGENESIS, PAX4_01, GCM_GSPT1, TGCTGCT_MIR15A_MIR16_MIR15B_MIR195_MIR424_MIR497, GOBP_FORMATION_OF_PRIMARY_GERM_LAYER, GOBP_NEGATIVE_REGULATION_OF_CELL_GROWTH, GOZGIT_ESR1_TARGETS_DN, GRAESSMANN_APOPTOSIS_BY_DOXORUBICIN_DN, GRAESSMANN_RESPONSE_TO_MC_AND_DOXORUBICIN_DN

GO Biological Process (38): MAPK cascade (GO:0000165), mesoderm formation (GO:0001707), negative regulation of cell-matrix adhesion (GO:0001953), ectoderm development (GO:0007398), negative regulation of cell population proliferation (GO:0008285), regulation of cell shape (GO:0008360), negative regulation of Schwann cell proliferation (GO:0010626), Schwann cell proliferation (GO:0014010), regulation of gliogenesis (GO:0014013), hippocampus development (GO:0021766), negative regulation of cell-cell adhesion (GO:0022408), actin cytoskeleton organization (GO:0030036), negative regulation of cell migration (GO:0030336), regulation of protein stability (GO:0031647), osteoblast proliferation (GO:0033687), negative regulation of osteoblast proliferation (GO:0033689), hippo signaling (GO:0035329), regulation of hippo signaling (GO:0035330), odontogenesis of dentin-containing tooth (GO:0042475), regulation of apoptotic process (GO:0042981), negative regulation of MAPK cascade (GO:0043409), cell-cell junction organization (GO:0045216), positive regulation of cell differentiation (GO:0045597), negative regulation of receptor signaling pathway via JAK-STAT (GO:0046426), positive regulation of stress fiber assembly (GO:0051496), regulation of cell cycle (GO:0051726), negative regulation of cell growth involved in contact inhibition (GO:0060243), lens fiber cell differentiation (GO:0070306), regulation of stem cell proliferation (GO:0072091), regulation of protein localization to nucleus (GO:1900180), regulation of organelle assembly (GO:1902115), positive regulation of protein localization to early endosome (GO:1902966), regulation of neural precursor cell proliferation (GO:2000177), positive regulation of early endosome to late endosome transport (GO:2000643), brain development (GO:0007420), cell differentiation (GO:0030154), regulation of cell population proliferation (GO:0042127), regulation of neurogenesis (GO:0050767)

GO Molecular Function (5): actin binding (GO:0003779), integrin binding (GO:0005178), signaling adaptor activity (GO:0035591), protein binding (GO:0005515), cytoskeletal protein binding (GO:0008092)

GO Cellular Component (21): nucleus (GO:0005634), nucleolus (GO:0005730), cytoplasm (GO:0005737), early endosome (GO:0005769), cytosol (GO:0005829), cytoskeleton (GO:0005856), plasma membrane (GO:0005886), adherens junction (GO:0005912), membrane (GO:0016020), lamellipodium (GO:0030027), filopodium (GO:0030175), cortical actin cytoskeleton (GO:0030864), filopodium membrane (GO:0031527), cleavage furrow (GO:0032154), ruffle membrane (GO:0032587), neuron projection (GO:0043005), cell body (GO:0044297), apical part of cell (GO:0045177), perinuclear region of cytoplasm (GO:0048471), ruffle (GO:0001726), cell projection (GO:0042995)

Reactome top-level categories

Rollup of top-7 pathways:

Category	Pathways
Fcgamma receptor (FCGR) dependent phagocytosis	1
RHO GTPase Effectors	1
Immune System	1
Signaling by Rho GTPases, Miro GTPases and RHOBTB3	1
Signaling by Rho GTPases	1
Innate Immune System	1
Signal Transduction	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	6
plasma membrane bounded cell projection	3
cell population proliferation	2
regulation of biological quality	2
intracellular membraneless organelle	2
cytoplasm	2
cell leading edge	2
cell projection membrane	2
intracellular signaling cassette	1
formation of primary germ layer	1
mesoderm morphogenesis	1
regulation of cell-matrix adhesion	1
cell-matrix adhesion	1
negative regulation of cell-substrate adhesion	1
tissue development	1
regulation of cell population proliferation	1
negative regulation of cellular process	1
regulation of cell morphogenesis	1
regulation of Schwann cell proliferation	1
Schwann cell proliferation	1
negative regulation of glial cell proliferation	1
glial cell proliferation	1
gliogenesis	1
regulation of neurogenesis	1
pallium development	1
limbic system development	1
anatomical structure development	1
negative regulation of cell adhesion	1
regulation of cell-cell adhesion	1
cell-cell adhesion	1
cytoskeleton organization	1
actin filament-based process	1
cell migration	1
regulation of cell migration	1
negative regulation of cell motility	1
negative regulation of cell population proliferation	1
osteoblast proliferation	1
regulation of osteoblast proliferation	1
intracellular signal transduction	1
hippo signaling	1

Protein interactions and networks

STRING

2458 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
NF2	WWC1	Q8IX03	979
NF2	NF1	P21359	971
NF2	IQCJ-SCHIP1	B3KU38	964
NF2	F8WDG0	F8WDG0	960
NF2	CD44	P16070	949
NF2	AMOT	Q4VCS5	939
NF2	SAV1	Q9H4B6	931
NF2	EZR	P15311	918
NF2	LATS1	O95835	909
NF2	FRMD6	Q96NE9	900
NF2	CTNNB1	P35222	881
NF2	NHERF1	O14745	870
NF2	CDH1	P12830	853
NF2	LATS2	Q9NRM7	834
NF2	LAYN	Q6UX15	828

IntAct

223 interactions, top by confidence:

A	B	Type	Score
IKBKG	IKBKB	psi-mi:“MI:0914”(association)	0.980
PIK3CA	PIK3R2	psi-mi:“MI:0914”(association)	0.900
NF2	AMOT	psi-mi:“MI:0915”(physical association)	0.880
NF2	AMOT	psi-mi:“MI:0403”(colocalization)	0.880
AMOT	NF2	psi-mi:“MI:0915”(physical association)	0.880
ATG5	ATG12	psi-mi:“MI:0914”(association)	0.800
NF2	AMOTL1	psi-mi:“MI:0914”(association)	0.730
CFTR	ESYT2	psi-mi:“MI:2364”(proximity)	0.710
NF2	HGS	psi-mi:“MI:2364”(proximity)	0.700
NF2	HGS	psi-mi:“MI:0915”(physical association)	0.700
HGS	NF2	psi-mi:“MI:0915”(physical association)	0.700
KDM1A	NF2	psi-mi:“MI:0915”(physical association)	0.670
MAP3K11	NF2	psi-mi:“MI:0915”(physical association)	0.650
NF2	MAP3K11	psi-mi:“MI:0407”(direct interaction)	0.650
MAP3K11	NF2	psi-mi:“MI:0217”(phosphorylation reaction)	0.650
NF2	GTPBP4	psi-mi:“MI:0915”(physical association)	0.610

BioGRID (527): NF2 (Affinity Capture-RNA), NF2 (Affinity Capture-RNA), NF2 (Affinity Capture-MS), NF2 (Affinity Capture-MS), SLC9A3R1 (Reconstituted Complex), LATS1 (Reconstituted Complex), LATS1 (Co-crystal Structure), LATS2 (Co-crystal Structure), AMOT (Reconstituted Complex), NF2 (Affinity Capture-Western), NF2 (Affinity Capture-Western), NF2 (Reconstituted Complex), NF2 (Affinity Capture-Western), LATS1 (Affinity Capture-Western), LATS1 (Co-localization)

ESM2 similar proteins: A0A1D5P556, A6H7I5, B0DOB5, F1M386, F1MSG6, F1PBJ0, G5EGS5, H2KZZ6, O13728, O95466, P21575, P23678, P26675, P27619, P35240, P39052, P39053, P39054, P39055, P46662, P48608, P50570, P55010, P59750, P78344, P79398, Q05193, Q08877, Q08DF4, Q15057, Q2KI89, Q54WH2, Q5R4L0, Q5R629, Q5R7J9, Q62448, Q6IVG4, Q6NXC0, Q6ZPF4, Q7PS12

Diamond homologs: B0WYY2, B2RYE5, O35763, O43491, O70318, P12264, P15311, P26038, P26040, P26041, P26042, P26043, P26044, P26045, P29074, P31976, P31977, P35240, P35241, P46150, P46662, P52962, P59750, P86232, Q12923, Q170J7, Q24564, Q29GR8, Q2HJ49, Q32LP2, Q58CU2, Q5FVG2, Q63648, Q66I42, Q7PS12, Q8BGS1, Q8HZQ5, Q8WY64, Q9H329, Q9HCM4

SIGNOR signaling

20 interactions.

A	Effect	B	Mechanism
PAK1	down-regulates	NF2	phosphorylation
PAK2	down-regulates	NF2	phosphorylation
PPP1CB	up-regulates	NF2	dephosphorylation
PRKACA	up-regulates	NF2	phosphorylation
PRKACA	down-regulates	NF2	phosphorylation
NF2	up-regulates	LATS1	binding
NF2	up-regulates	LATS2	binding
NEDD4L	“up-regulates activity”	NF2	ubiquitination
NF2	“up-regulates activity”	LATS1/2	binding
PAK	down-regulates	NF2	phosphorylation
DCAF1	“down-regulates quantity by destabilization”	NF2	binding
Cullin4-RBX1-DDB1	“down-regulates quantity by destabilization”	NF2	polyubiquitination
TNIK	“up-regulates activity”	NF2	phosphorylation
PRKCA	“down-regulates activity”	NF2	phosphorylation
NF2	“up-regulates activity”	STK3	binding
NF2	“up-regulates activity”	STK4	binding
NF2	“up-regulates activity”	STK3/4	binding

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 122 IntAct physical interaction partners (hypergeometric vs the genome-wide background, BH-FDR, gene-set size 15–500, ranked by fold). A functional readout of the neighbourhood — distinct from this gene’s own memberships above, and biased toward well-studied / hub proteins, so read it as themes rather than proof.

Reactome pathways:

Pathway	Partners	Fold	FDR
Constitutive Signaling by EGFRvIII	6	49.2×	6e-07
Signaling by ERBB2 ECD mutants	6	46.3×	6e-07
Constitutive Signaling by Ligand-Responsive EGFR Cancer Variants	6	39.4×	1e-06
Tie2 Signaling	5	34.5×	2e-05
Signaling by FLT3 fusion proteins	5	32.8×	2e-05
Signaling by phosphorylated juxtamembrane, extracellular and kinase domain KIT mutants	5	29.8×	2e-05
Signaling by ERBB2 KD Mutants	6	29.2×	3e-06
Signaling by ERBB2 TMD/JMD mutants	5	27.4×	3e-05

GO biological processes:

GO term	Partners	Fold	FDR
positive regulation of stress fiber assembly	5	13.8×	8e-03
MAPK cascade	7	9.5×	3e-03

Disease & clinical

Cancer significance

From intOGen — cancer-driver classification: loss-of-function (tumor-suppressor-like) across 10 cancer types — CCRCC, CESC, HCC, HNSC, MEL, OVT, PAAD, PLMESO, PRCC, RCC.

Clinical variants and AI predictions

ClinVar

2555 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	260
Likely pathogenic	51
Uncertain significance	1188
Likely benign	771
Benign	61

Top pathogenic / likely-pathogenic (30)

Variant ID	HGVS	Classification
1041202	NC_000022.10:g.(?30050636)(30050946_?)del	Pathogenic
1069587	NM_000268.4(NF2):c.375_376del (p.Gln125fs)	Pathogenic
1070353	NM_000268.4(NF2):c.648_649dup (p.Tyr217fs)	Pathogenic
1070378	NC_000022.10:g.(?29999982)(30090797_?)del	Pathogenic
1070379	NC_000022.10:g.(?29999988)(30090791_?)del	Pathogenic
1072529	NC_000022.10:g.(?_30051485)_30051628del	Pathogenic
1072589	NM_000268.4(NF2):c.240+2T>C	Pathogenic
1072590	NM_000268.4(NF2):c.241-2A>G	Pathogenic
1074395	NM_000268.4(NF2):c.481G>T (p.Gly161Ter)	Pathogenic
1074791	NM_000268.4(NF2):c.457dup (p.Tyr153fs)	Pathogenic
1074869	NM_000268.4(NF2):c.320_321del (p.Glu107fs)	Pathogenic
1075085	NM_000268.4(NF2):c.1606C>T (p.Gln536Ter)	Pathogenic
1075185	NM_000268.4(NF2):c.1621G>T (p.Glu541Ter)	Pathogenic
1076273	NM_000268.4(NF2):c.1198C>T (p.Gln400Ter)	Pathogenic
1297592	NM_000268.4(NF2):c.663C>G (p.Tyr221Ter)	Pathogenic
1299301	NM_000268.4(NF2):c.770_784del (p.Pro257_Ile261del)	Pathogenic
1319646	NM_000268.4(NF2):c.448-1G>A	Pathogenic
1323362	NM_000268.4(NF2):c.114+1G>T	Pathogenic
1354127	NM_000268.4(NF2):c.1341-1G>A	Pathogenic
1354329	NM_000268.4(NF2):c.1627_1628del (p.Lys543fs)	Pathogenic
1368800	NM_000268.4(NF2):c.1051del (p.Arg351fs)	Pathogenic
1389374	NM_000268.4(NF2):c.999+2T>G	Pathogenic
1422073	NM_000268.4(NF2):c.1490del (p.Ser497fs)	Pathogenic
1429536	NM_000268.4(NF2):c.1575-6C>A	Pathogenic
1430714	NM_000268.4(NF2):c.363+1G>T	Pathogenic
1451331	NM_000268.4(NF2):c.1074del (p.Arg359fs)	Pathogenic
1451707	NM_000268.4(NF2):c.229del (p.Met77fs)	Pathogenic
1452085	NM_000268.4(NF2):c.1022del (p.Arg341fs)	Pathogenic
1452260	NC_000022.10:g.(?29999988)(30032875_?)del	Pathogenic
1452573	NM_000268.4(NF2):c.302_303del (p.Tyr101fs)	Pathogenic

SpliceAI

3483 predictions. Top by Δscore:

Variant	Effect	Δscore
22:29654651:TTCCA:T	acceptor_loss	1.0000
22:29654653:CCA:C	acceptor_loss	1.0000
22:29654655:A:AG	acceptor_gain	1.0000
22:29654655:AGTA:A	acceptor_loss	1.0000
22:29654655:AGTAT:A	acceptor_gain	1.0000
22:29654656:G:GA	acceptor_gain	1.0000
22:29654656:GT:G	acceptor_gain	1.0000
22:29654656:GTA:G	acceptor_gain	1.0000
22:29654656:GTAT:G	acceptor_gain	1.0000
22:29654656:GTATG:G	acceptor_gain	1.0000
22:29654722:AAGGG:A	donor_loss	1.0000
22:29654723:AGGG:A	donor_loss	1.0000
22:29654724:GG:G	donor_gain	1.0000
22:29654724:GGGTA:G	donor_loss	1.0000
22:29654725:GG:G	donor_gain	1.0000
22:29654725:GGTA:G	donor_loss	1.0000
22:29654726:G:GG	donor_gain	1.0000
22:29654727:T:A	donor_loss	1.0000
22:29655591:TAGGT:T	acceptor_loss	1.0000
22:29655593:G:GC	acceptor_loss	1.0000
22:29658183:CCACA:C	acceptor_loss	1.0000
22:29658186:CAGG:C	acceptor_loss	1.0000
22:29658187:A:C	acceptor_loss	1.0000
22:29658187:AG:A	acceptor_gain	1.0000
22:29658188:GG:G	acceptor_gain	1.0000
22:29658234:GAT:G	donor_gain	1.0000
22:29658264:G:GT	donor_gain	1.0000
22:29661336:GGAG:G	donor_gain	1.0000
22:29661337:GAGG:G	donor_gain	1.0000
22:29661339:GGTA:G	donor_loss	1.0000

AlphaMissense

3970 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
22:29636809:A:T	E58V	1.000
22:29636856:T:A	W74R	1.000
22:29636856:T:C	W74R	1.000
22:29639154:C:A	P102H	1.000
22:29639175:T:C	L109P	1.000
22:29642235:T:C	C133R	1.000
22:29642236:G:A	C133Y	1.000
22:29642237:C:G	C133W	1.000
22:29642257:T:C	L140P	1.000
22:29642260:T:C	L141P	1.000
22:29642263:C:A	A142D	1.000
22:29642271:G:C	A145P	1.000
22:29642275:T:A	V146D	1.000
22:29654718:C:A	P170Q	1.000
22:29655627:T:A	W184R	1.000
22:29655627:T:C	W184R	1.000
22:29655628:G:C	W184S	1.000
22:29655629:G:C	W184C	1.000
22:29655629:G:T	W184C	1.000
22:29658208:T:G	Y207D	1.000
22:29658212:T:C	L208P	1.000
22:29658221:C:A	A211D	1.000
22:29658253:T:C	F222L	1.000
22:29658255:T:A	F222L	1.000
22:29658255:T:G	F222L	1.000
22:29661230:T:C	L234P	1.000
22:29661232:G:A	G235R	1.000
22:29661232:G:C	G235R	1.000
22:29661233:G:A	G235E	1.000
22:29661247:G:A	G240R	1.000

dbSNP variants (sampled 300 via entrez): RS1000005532 (22:29628308 T>C,G), RS1000050568 (22:29692743 G>A), RS1000079254 (22:29636077 A>G), RS1000178580 (22:29608630 C>T), RS1000197871 (22:29653839 G>A), RS1000240025 (22:29621528 G>A,T), RS1000252083 (22:29608301 CAG>C), RS1000260812 (22:29650405 C>T), RS1000277452 (22:29650014 A>G), RS1000280774 (22:29695281 C>G,T), RS1000295988 (22:29671400 G>A), RS1000331140 (22:29660955 C>G,T), RS1000392617 (22:29657093 A>C,G), RS1000516929 (22:29633962 C>G,T), RS1000551534 (22:29679331 T>C)

Disease associations

OMIM: gene MIM:607379 | disease phenotypes: MIM:101000, MIM:607174, MIM:162200, MIM:162091, MIM:174050

GenCC curated gene-disease

Disease	Classification	Inheritance
NF2-related schwannomatosis	Definitive	Autosomal dominant
familial meningioma	Limited	Unknown

ClinGen Gene-Disease Validity (1)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

Disease	Classification	Inheritance
NF2-related schwannomatosis	Definitive	AD

Mondo (13): NF2-related schwannomatosis (MONDO:0007039), hereditary neoplastic syndrome (MONDO:0015356), familial meningioma (MONDO:0011789), neurofibromatosis type 1 (MONDO:0018975), SMARCB1-related schwannomatosis (MONDO:0024517), autosomal dominant polycystic liver disease (MONDO:0000447), meningioma (MONDO:0016642), acoustic neuroma (MONDO:0001569), breast cancer (MONDO:0007254), choroid plexus carcinoma (MONDO:0016718), ependymoma (MONDO:0016698), spindle cell sarcoma (MONDO:0002927), nervous system cancer (MONDO:0005872)

Orphanet (10): Inherited cancer-predisposing syndrome (Orphanet:140162), Full NF2-related schwannomatosis (Orphanet:637), Familial multiple meningioma (Orphanet:263662), Neurofibromatosis type 1 (Orphanet:636), Full schwannomatosis (Orphanet:93921), Isolated polycystic liver disease (Orphanet:2924), Meningioma (Orphanet:2495), Vestibular schwannoma (Orphanet:252175), Choroid plexus carcinoma (Orphanet:251899), Ependymoma (Orphanet:251636)

HPO phenotypes

137 total (30 of 137 shown, HPO-id order):

HPO	Term
HP:0000006	Autosomal dominant inheritance
HP:0000020	Urinary incontinence
HP:0000044	Hypogonadotropic hypogonadism
HP:0000131	Uterine leiomyoma
HP:0000141	Amenorrhea
HP:0000238	Hydrocephalus
HP:0000360	Tinnitus
HP:0000365	Hearing impairment
HP:0000407	Sensorineural hearing impairment
HP:0000478	Abnormality of the eye
HP:0000518	Cataract
HP:0000520	Proptosis
HP:0000572	Visual loss
HP:0000587	Abnormal optic nerve morphology
HP:0000602	Ophthalmoplegia
HP:0000618	Blindness
HP:0000646	Amblyopia
HP:0000651	Diplopia
HP:0000712	Emotional lability
HP:0000751	Personality changes
HP:0000763	Sensory neuropathy
HP:0000802	Impotence
HP:0000870	Increased circulating prolactin concentration
HP:0000953	Hyperpigmentation of the skin
HP:0000957	Cafe-au-lait spot
HP:0000997	Axillary freckling
HP:0001067	Neurofibroma
HP:0001085	Papilledema
HP:0001250	Seizure
HP:0001251	Ataxia

GWAS associations

1 associations (top):

Study	Trait	p-value
GCST000555_7	Carotid atherosclerosis in HIV infection	2.000000e-06

MeSH disease descriptors (8)

Descriptor	Name	Tree numbers
D004806	Ependymoma	C04.557.465.625.600.380.290; C04.557.470.670.380.290; C04.557.580.625.600.380.290
D008579	Meningioma	C04.557.580.520; C04.557.645.520; C04.588.614.250.580.500; C10.551.240.500.500
D009386	Neoplastic Syndromes, Hereditary	C04.700; C16.320.700
D009423	Nervous System Neoplasms	C04.588.614; C10.551
D009456	Neurofibromatosis 1	C04.557.580.600.580.590.650; C04.700.631.650; C10.562.600.500; C10.574.500.549.400; C10.668.829.675; C16.320.400.560.400; C16.320.700.633.650
D009464	Neuroma, Acoustic	C04.557.465.625.650.595.610; C04.557.580.600.610.595.610; C04.557.580.625.650.595.610; C04.588.614.300.015; C04.588.614.596.240.015; C09.218.807.800.675; C09.647.675; C10.292.225.750; C10.292.910.600
C562943	Choroid Plexus Carcinoma (supp.)
C537443	Meningioma, familial (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

Clinical evidence (CIViC)

Drug × variant × indication: 3 predictive associations from 3 curated evidence items; also 1 diagnostic, 1 predisposing.

Variant	Therapy	Indication	Effect	Level	CIViC
NF2 K159fs	Temsirolimus	Breast Cancer	Sensitivity/Response	CIViC C	EID715
NF2 Y177fs	Carboplatin + Cisplatin	Peritoneal Mesothelioma	Resistance	CIViC C	EID649
NF2 Loss	Selumetinib	Thyroid Gland Carcinoma	Sensitivity/Response	CIViC D	EID1742

PharmGKB: 1 entry (VIP=true, CPIC=false)

CTD chemical–gene interactions

47 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
zinc chloride	affects reaction, increases expression, decreases expression	2
Arsenic Trioxide	decreases expression	2
FR900359	affects phosphorylation	1
dicrotophos	increases expression	1
geldanamycin	increases expression	1
alpha-pinene	affects cotreatment, decreases expression, increases abundance	1
tris(1,3-dichloro-2-propyl)phosphate	decreases expression	1
sodium arsenite	decreases expression	1
cobaltous chloride	decreases expression	1
4-hydroxy-2-nonenal	decreases expression	1
coumarin	decreases phosphorylation	1
methacrylaldehyde	decreases expression, increases abundance, affects cotreatment	1
evodiamine	decreases expression	1
polyhexamethyleneguanidine	affects expression	1
di-n-butylphosphoric acid	affects expression	1
torcetrapib	increases expression	1
jinfukang	decreases expression, increases reaction	1
(+)-JQ1 compound	decreases response to substance	1
Sevoflurane	increases expression, increases reaction	1
Sunitinib	decreases expression	1
Acrolein	affects cotreatment, decreases expression, increases abundance	1
Air Pollutants	affects cotreatment, decreases expression, increases abundance	1
Amiodarone	increases expression	1
Amphotericin B	decreases expression	1
Cisplatin	decreases expression, increases reaction	1
Doxorubicin	decreases expression	1
Dust	decreases expression	1
Hydrogen Peroxide	decreases expression	1
Ivermectin	decreases expression	1
Lipopolysaccharides	increases expression, increases reaction	1

Cellosaurus cell lines

87 cell lines: 74 cancer cell line, 7 transformed cell line, 5 telomerase immortalized cell line, 1 induced pluripotent stem cell

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_0455	NCI-H292	Cancer cell line	Female
CVCL_1054	8505C	Cancer cell line	Female
CVCL_1067	ACHN	Cancer cell line	Male
CVCL_1112	CAL-62	Cancer cell line	Female
CVCL_1518	NCI-H2052	Cancer cell line	Male
CVCL_1785	VA-ES-BJ	Cancer cell line	Male
CVCL_1959	Ben-Men-1	Telomerase immortalized cell line	Female
CVCL_2104	LOU-NH91	Cancer cell line	Female
CVCL_2794	SKG-II	Cancer cell line	Female
CVCL_4J76	SKG-II/LUC	Cancer cell line	Female

Clinical trials (associated diseases)

189 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT04081701	PHASE4	RECRUITING	68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors.
NCT04386642	PHASE4	UNKNOWN	Tranexamic Acid Reduce Blood Loss in Meningioma Resection
NCT06377371	PHASE4	RECRUITING	Feasibility of Intraoperative Tracing of Meningioma Using [Cu64]DOTATATE
NCT00517959	PHASE3	UNKNOWN	SCRT Versus Conventional RT in Children and Young Adults With Low Grade and Benign Brain Tumors
NCT01655927	PHASE3	UNKNOWN	Efficacy of Tranexamic Acid in Brain Tumor Resections
NCT03015701	PHASE3	COMPLETED	S9005 Mifepristone in Meningioma
NCT03558516	PHASE3	COMPLETED	Magnesium and Intraoperative Blood Loss in Meningioma Surgery
NCT04305470	PHASE3	COMPLETED	Gleolan for Visualization of Newly Diagnosed or Recurrent Meningioma
NCT00004437	PHASE2	COMPLETED	Phase II Study of the Multichannel Auditory Brain Stem Implant for Deafness Following Surgery for Neurofibromatosis 2
NCT00911248	PHASE2	TERMINATED	PTC299 for Treatment of Neurofibromatosis Type 2
NCT00973739	PHASE2	COMPLETED	Lapatinib Study for Children and Adults With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors
NCT01125046	PHASE2	COMPLETED	Bevacizumab in Treating Patients With Recurrent or Progressive Meningiomas
NCT01207687	PHASE2	COMPLETED	Bevacizumab for Symptomatic Vestibular Schwannoma in Neurofibromatosis Type 2 (NF2)
NCT01345136	PHASE2	TERMINATED	Study of RAD001 for Treatment of NF2-related Vestibular Schwannoma
NCT01419639	PHASE2	COMPLETED	Phase II Study of Everolimus (RAD001) in Children and Adults With Neurofibromatosis Type 2
NCT01490476	PHASE2	COMPLETED	Efficacy and Safety Study of RAD001 in the Growth of the Vestibular Schwannoma(s) in Neurofibromatosis 2 (NF2) Patients
NCT01767792	PHASE2	COMPLETED	Phase 2 Study of Bevacizumab in Children and Young Adults With NF 2 and Progressive Vestibular Schwannomas
NCT02104323	PHASE2	COMPLETED	Endostatin Study for Patients With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors
NCT02129647	PHASE2	COMPLETED	Study of Axitinib in Patients With Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas
NCT02831257	PHASE2	COMPLETED	AZD2014 In NF2 Patients With Progressive or Symptomatic Meningiomas
NCT02934256	PHASE2	COMPLETED	Icotinib Study for Patients With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors
NCT03079999	PHASE2	ACTIVE_NOT_RECRUITING	Study of Aspirin in Patients With Vestibular Schwannoma
NCT03095248	PHASE2	TERMINATED	Trial of Selumetinib in Patients With Neurofibromatosis Type II Related Tumors
NCT04283669	PHASE2	COMPLETED	Phase 2 Clinical Trial of Crizotinib for Children and Adults With Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas
NCT04374305	PHASE2	RECRUITING	Innovative Trial for Understanding the Impact of Targeted Therapies in NF2-Related Schwannomatosis (INTUITT-NF2)
NCT00003483	PHASE2	TERMINATED	Antineoplaston Therapy in Treating Patients With Meningioma
NCT00589784	PHASE2	COMPLETED	Phase II Trial of Sunitinib (SU011248) in Patients With Recurrent or Inoperable Meningioma
NCT00706810	PHASE2	COMPLETED	Combination of Hydroxyurea and Verapamil for Refractory Meningiomas
NCT00859040	PHASE2	COMPLETED	Monthly SOM230C for Recurrent or Progressive Meningioma
NCT01967823	PHASE2	COMPLETED	T Cell Receptor Immunotherapy Targeting NY-ESO-1 for Patients With NY-ESO-1 Expressing Cancer
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