Attiah Bashnona, Alewine Garrett, Easter Mary-Kate, Coover Robert A, Fahrenholtz Cale D
Department of Basic Pharmaceutical Sciences, Fred Wilson School of Pharmacy, High Point University, High Point, NC 27268, USA.
Pharmaceutics. 2024 Mar 7;16(3):371. doi: 10.3390/pharmaceutics16030371.
Neurofibromatosis Type 1 (NF1) is a common neurogenic condition characterized by heterozygous loss of function mutations in the neurofibromin gene. NF1 patients are susceptible to the development of neurofibromas, including plexiform neurofibromas (pNFs), which occurs in about half of all cases. Plexiform neurofibroma are benign peripheral nerve sheath tumors originating from Schwann cells after complete loss of neurofibromin; they can be debilitating and also transform into deadly malignant peripheral nerve sheath tumors (MPNSTs). Here, our data indicates that silver nanoparticles (AgNPs) may be useful in the treatment of pNFs. We assessed the cytotoxicity of AgNPs using pNF cells and Schwann cells derived from the same NF1 patient. We found that AgNPs are selectively cytotoxic to pNF cells relative to isogenic Schwann cells. We then examined the role of neurofibromin expression on AgNP-mediated cytotoxicity; restoration of neurofibromin expression in pNF cells decreased sensitivity to AgNP, and knockdown of neurofibromin in isogenic Schwann cells increased sensitivity to AgNP, outlining a correlation between neurofibromin expression and AgNP-mediated cytotoxicity. AgNP was able to selectively remove pNF cells from a co-culture with patient-matched Schwann cells. Therefore, AgNPs represent a new approach for clinical management of NF1-associated pNF to address significant clinical need.
1型神经纤维瘤病(NF1)是一种常见的神经源性疾病,其特征是神经纤维瘤蛋白基因发生杂合性功能丧失突变。NF1患者易患神经纤维瘤,包括丛状神经纤维瘤(pNFs),约半数病例会出现这种情况。丛状神经纤维瘤是良性周围神经鞘瘤,由神经纤维瘤蛋白完全缺失后的施万细胞起源;它们可能使人衰弱,还会恶变为致命的恶性周围神经鞘瘤(MPNSTs)。在此,我们的数据表明银纳米颗粒(AgNPs)可能对pNFs的治疗有用。我们使用来自同一名NF1患者的pNF细胞和施万细胞评估了AgNPs的细胞毒性。我们发现,相对于同基因的施万细胞,AgNPs对pNF细胞具有选择性细胞毒性。然后,我们研究了神经纤维瘤蛋白表达在AgNP介导的细胞毒性中的作用;pNF细胞中神经纤维瘤蛋白表达的恢复降低了对AgNP的敏感性,而在同基因施万细胞中敲低神经纤维瘤蛋白则增加了对AgNP的敏感性,这揭示了神经纤维瘤蛋白表达与AgNP介导的细胞毒性之间的相关性。AgNP能够从与患者匹配的施万细胞的共培养物中选择性地清除pNF细胞。因此,AgNPs代表了一种针对NF1相关pNF临床管理的新方法,以满足重大的临床需求。