ZNF100

Neurofibromin UniProt accession P21359

Stimulates the GTPase activity of Ras. NF1 shows greater affinity for Ras GAP, but lower specific activity. May be a regulator of Ras activity

Source: UniProt

Interacts with HTR6 (PubMed:23027611). Interacts with SPRED2 (PubMed:34626534)

Source: UniProt
Nucleus, Nucleus, nucleolus, Cell membrane
Source: UniProt

Detected in brain, peripheral nerve, lung, colon and muscle

Source: UniProt

Binds phospholipids via its C-terminal CRAL-TRIO domain. Binds primarily glycerophospholipids with monounsaturated C18:1 and/or C16:1 fatty acid moieties and a phosphatidylethanolamine or phosphatidylcholine headgroup. Has lesser affinity for lipids containing phosphatidylserine and phosphatidylinositol

Source: UniProt
  • Neurofibromatosis 1 (NF1)

    A disease characterized by patches of skin pigmentation (cafe-au-lait spots), Lisch nodules of the iris, tumors in the peripheral nervous system and fibromatous skin tumors. Individuals with the disorder have increased susceptibility to the development of benign and malignant tumors.

  • Leukemia, juvenile myelomonocytic (JMML)

    An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages.

  • Watson syndrome (WTSN)

    A syndrome characterized by the presence of pulmonary stenosis, cafe-au-lait spots, and intellectual disability. It is considered as an atypical form of neurofibromatosis.

  • Familial spinal neurofibromatosis (FSNF)

    Considered to be an alternative form of neurofibromatosis, showing multiple spinal tumors.

  • Neurofibromatosis-Noonan syndrome (NFNS)

    Characterized by manifestations of both NF1 and Noonan syndrome (NS). NS is a disorder characterized by dysmorphic facial features, short stature, hypertelorism, cardiac anomalies, deafness, motor delay, and a bleeding diathesis.

  • Colorectal cancer (CRC)

    A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history.

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 ZNF100, 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: 266

NCT ID Condition Brief Title Phase Status
NCT02811718 Neurofibromatosis 2 Resiliency Training for Patients With NF2 Via Videoconferencing With Skype NA COMPLETED
NCT04534283 Cancer, Cancer Metastatic, BRAF V600E, MEK1 Gene Mutation, MEK2 Gene Mutation, ERK Mutation, RAF1 Gene Mutation A Basket Trial of an ERK1/2 Inhibitor (LY3214996) in Combination With Abemaciclib. PHASE2 TERMINATED
NCT04800822 Solid Tumor PF-07284892 in Participants With Advanced Solid Tumors PHASE1 TERMINATED
NCT00111384 Neurofibromatosis Type 1, Legius Syndrome Study of Disease Severity in Adults With Neurofibromatosis Type 1 (NF1) N/A 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
NCT01338857 neurofibromatosis1 (NF1), Recurrent or Progressive Optic Pathway Gliomas (OPG), Recurrent or Progressive Low-grade Glioma Sorafenib in Children and Young Adults With Recurrent or Progressive Low-Grade Astrocytomas PHASE2 TERMINATED
NCT05831995 Advanced Solid Tumor, RAS Mutation, RAF Mutation, NF1 Mutation Safety and Effectiveness of ABM-168 in Adults with Advanced Solid Tumors. PHASE1 TERMINATED
NCT05331105 Neurofibromatosis 1, Plexiform Neurofibromas HL-085 in Adults With Neurofibromatosis Type 1 (NF1) and Inoperable Plexiform Neurofibromas PHASE2 RECRUITING
NCT06188741 Neurofibromatosis 1, Plexiform Neurofibroma Selumetinib for the Prevention of Plexiform Neurofibroma Growth in NF Type 1 PHASE2 RECRUITING