ZNF280B

Merlin UniProt accession 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. 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

Source: UniProt

Interacts with NHERF1, HGS and AGAP2. Interacts with LAYN (By similarity). 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 (PubMed:21167305). Interacts with SCHIP1; the interaction is direct (PubMed:10669747)

Source: UniProt
Cell projection, filopodium membrane — Peripheral membrane protein, Cell projection, ruffle membrane — Peripheral membrane protein, Nucleus Cytoplasm, perinuclear region, Cytoplasmic granule Cytoplasm, perinuclear region, Cytoplasmic granule Nucleus, Cell projection, filopodium membrane — Peripheral membrane protein, Cell projection, ruffle membrane — Peripheral membrane protein, Cytoplasm, perinuclear region, Cytoplasmic granule, Cytoplasm, cytoskeleton
Source: UniProt

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

Source: UniProt
  • Schwannomatosis, vestibular (SWNV)

    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.

  • Mesothelioma, malignant (MESOM)

    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.

Source: UniProt

Click a pathway to open the interactive Reactome viewer.

Pathway list and interactive viewer: Reactome

Synthetic Lethal Network

Genes with an experimentally identified or computationally predicted synthetic-lethal relationship to ZNF280B, aggregated across our SSL data sources. Click any partner node to view that gene’s page.

Nodes and edges are coloured by the SSL data source. Partners appearing in more than one source are shown in grey.

BioGRID SLOrth SynLethDB MexDrugs Multi-source
Sources: BioGRID, SLOrth, SynLethDB, MexDrugs

Clinical Trials

Total Trials Found: 64

NCT ID Condition Brief Title Phase Status
NCT02811718 Neurofibromatosis 2 Resiliency Training for Patients With NF2 Via Videoconferencing With Skype NA COMPLETED
NCT05521048 Neurofibromatosis Type 2 Doxycycline in Cutaneous Schwannoma (NF2) PHASE1, PHASE2 ACTIVE_NOT_RECRUITING
NCT00911248 Neurofibromatosis 2 PTC299 for Treatment of Neurofibromatosis Type 2 PHASE2 TERMINATED
NCT06104488 Refractory Cancer, CNS Tumors, CNS Tumor, Adult, CNS Tumor, Childhood, MAP Kinase Family Gene Mutation, NF1, Plexiform Neurofibroma, Low-grade Glioma, Optic Pathway Gliomas, Neuroblastoma, Primary Brain Tumor, Solid Tumor, Solid Tumor, Adult, Solid Carcinoma, Central Nervous System Tumor A Study of Avutometinib for People With Solid Tumor Cancers PHASE1 RECRUITING
NCT02298270 Neurofibromatosis, Neurofibromatosis I, Neurofibromatosis 2, Schwannomatosis Resiliency Training for Patients With Neurofibromatosis Via Videoconferencing With Skype NA COMPLETED
NCT04283669 Neurofibromatosis 2, Progressive Vestibular Schwannoma (VS) Phase 2 Clinical Trial of Crizotinib for Children and Adults With Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas PHASE2 COMPLETED
NCT06834438 NF2 Deficiency Gene Therapy for Neurofibromatosis Type 2 (NF2) with ST002 NA NOT_YET_RECRUITING
NCT02523014 Intracranial Meningioma, Recurrent Meningioma, NF2 Gene Mutation Vismodegib, FAK Inhibitor GSK2256098, Capivasertib, and Abemaciclib in Treating Patients With Progressive Meningiomas PHASE2 RECRUITING
NCT02102256 Profound Bilateral Deafness Due to, Bilateral Cochlear Aplasia, Bilateral Cochlear Nerve Deficiency, Bilateral Cochlear Ossification Secondary to Meningitis A Safety Study of the Auditory Brainstem Implant for Pediatric Profoundly Deaf Patients PHASE1 COMPLETED
NCT04857372 Mesothelioma A Phase I Study of IAG933 in Patients With Advanced Mesothelioma and Other Solid Tumors PHASE1 ACTIVE_NOT_RECRUITING