ArticleBrain : a journal of neurology2013
Loss of SOX10 function contributes to the phenotype of human Merlin-null schwannoma cells.
Article in Brain : a journal of neurology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 42 citations in OpenAlex.
- Myelination and Remyelination in Peripheral Nerve Injury: Mechanisms, Regulation, and Therapeutic Perspectives.MedComm · 2026Review
- Culture and gene expression profiling of Schwann cells from human sural nerve.Communications biology · 2026Article
- Comparative neuroprotective and cognitive effects of human umbilical cord mesenchymal stem cells, secretome, and exosomes in a rat model of pilocarpine-induced epilepsy.Veterinary world · 2026Article
- Vestibular schwannoma: genetic and epigenetic mechanisms, hearing loss, and emerging therapies.Journal of neuro-oncology · 2026Review
- Current molecular understanding of central nervous system schwannomas.Acta neuropathologica communications · 2025Review
- PI4P-mediated solid-like Merlin condensates orchestrate Hippo pathway regulation.Science (New York, N.Y.) · 2024Article
- Article
- Clinical Significance of SOX10 Expression in Human Pathology.Current issues in molecular biology · 2023Review
- Identification of immune-related candidate biomarkers in plasma of patients with sporadic vestibular schwannoma.Science advances · 2023Article
- Decoding key cell sub-populations and molecular alterations in glioblastoma at recurrence by single-cell analysis.Acta neuropathologica communications · 2023Article
- Recent knowledge of NFATc4 in oncogenesis and cancer prognosis.Cancer cell international · 2022Review
- Cell Shape and Matrix Stiffness Impact Schwann Cell Plasticity via YAP/TAZ and Rho GTPases.International journal of molecular sciences · 2021Article
- Meta-Analysis Reveals Transcription Factor Upregulation in Cells of Injured Mouse Sciatic Nerve.Frontiers in cellular neuroscience · 2021Article
- Single-Cell Regulatory Network Inference and Clustering Identifies Cell-Type Specific Expression Pattern of Transcription Factors in Mouse Sciatic Nerve.Frontiers in cellular neuroscience · 2021Article
- SOX10-regulated promoter use defines isoform-specific gene expression in Schwann cells.BMC genomics · 2020Article
- Sex-Determining Region Y Chromosome-Related High-Mobility-Group Box 10 in Cancer: A Potential Therapeutic Target.Frontiers in cell and developmental biology · 2020Review
- Whole Genome Sequencing Identifies Key Genes in Spinal Schwannoma.Frontiers in genetics · 2020Article
- Sox2 expression in Schwann cells inhibits myelinationDevelopment (Cambridge, England) · 2017Article
- Merlin controls the repair capacity of Schwann cells after injury by regulating Hippo/YAP activity.The Journal of cell biology · 2017Article
- Text mining and network analysis of molecular interaction in non-small cell lung cancer by using natural language processing.Molecular biology reports · 2014Article
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Authors and funding
9 authors at 3 institutions in 3 countries.
Funding
Abstract
Loss of the Merlin tumour suppressor causes abnormal de-differentiation and proliferation of Schwann cells and formation of schwannoma tumours in patients with neurofibromatosis type 2. Within the mature peripheral nerve the normal development, differentiation and maintenance of myelinating and non-myelinating Schwann cells is regulated by a network of transcription factors that include SOX10, OCT6 (now known as POU3F1), NFATC4 and KROX20 (also known as Egr2). We have examined for the first time how their regulation of Schwann cell development is disrupted in primary human schwannoma cells. We find that induction of both KROX20 and OCT6 is impaired, whereas enforced expression of KROX20 drives both myelin gene expression and cell cycle arrest in Merlin-null cells. Importantly, we show that human schwannoma cells have reduced expression of SOX10 protein and messenger RNA. Analysis of mouse SOX10-null Schwann cells shows they display many of the characteristics of human schwannoma cells, including increased expression of platelet derived growth factor receptor beta (PDGFRB) messenger RNA and protein, enhanced proliferation, increased focal adhesions and schwannoma-like morphology. Correspondingly, reintroduction of SOX10 into human Merlin-null cells restores the ability of these cells to induce KROX20 and myelin protein zero (MPZ), localizes NFATC4 to the nucleus, reduces cell proliferation and suppresses PDGFRB expression. Thus, we propose that loss of the SOX10 protein, which is vital for normal Schwann cell development, is also key to the pathology of Merlin-null schwannoma tumours.
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