Plexiform neurofibromas (PNFs) are a prevalent and severe phenotype associated with NF1, characterized by a high teratogenic rate and potential for malignant transformation. The growth and recurrence of PNFs are attributed to aberrant proliferation and migration of Nf1-deficient Schwann cells. Protein tyrosine phosphatase receptor S (PTPRS) is believed to modulate cell migration and invasion by inhibiting the EMT process in NF1-derived malignant peripheral nerve sheath tumors.
View Article and Find Full Text PDFZhongguo Xiu Fu Chong Jian Wai Ke Za Zhi
January 2024
Objective: To summarize the gene therapy strategies for neurofibromatosis type 1 (NF1) and related research progress.
Methods: The recent literature on gene therapy for NF1 at home and abroad was reviewed. The structure and function of the NF1 gene and its mutations were analyzed, and the current status as well as future prospects of the transgenic therapy and gene editing strategies were summarized.
To evaluate the effectiveness of augmented reality (AR) game based on -back training paradigm as a training tool for working memory (WM) of Chinese healthy older adults. One hundred eighteen older adults self-assessed as healthy were included in this study. Individuals were randomly divided into an intervention group ( = 57) and a control group ( = 61).
View Article and Find Full Text PDF(1) Background: malignant peripheral nerve sheath tumours (MPNSTs) are aggressive Schwann cell-derived sarcomas with dismal prognoses. Previous studies have shown that nuclear receptor corepressor 2 (NCOR2) plays a vital role in neurodevelopment and in various tumours. However, the impact of NCOR2 on the progression of MPNST remains unclear.
View Article and Find Full Text PDFZhongguo Xiu Fu Chong Jian Wai Ke Za Zhi
September 2022
Objective: To summarize current widely-used therapies for cutaneous neurofibroma (cNF) and related research progress.
Methods: Based on extensive investigation of domestic and foreign research, the existing treatment of cNF, including the indications, effectiveness and trials of targeted drugs were reviewed.
Results: cNF is a hallmark feature of neurofibromatosis type 1 and has a dramatic negative impact on patient appearance and quality of life.
Trametinib has been used in neurofibromatosis type 1 (NF1) patients, especially those with unresectable nerve tumors, but no systematic review based on the latest studies has been published. We conducted this meta-analysis to evaluate the effectiveness and safety of trametinib in treating NF1-related nerve tumors. Original articles reporting the efficacy and safety of trametinib in NF1 patents were identified in PubMed, EMBASE, and Web of Science up to 1 June 2022.
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