SMS-CTR人胚胎性横纹肌肉瘤细胞株（ERMS）BioVector™ human embryonal rhabdomyosarcoma cell line BioVector NTCC保藏中心

BioVector™ SMS-CTR 细胞系 是一种广泛使用的人类癌细胞系，它专门来源于一名患有**胚胎性横纹肌肉瘤（ERMS）**的患者。横纹肌肉瘤（RMS）是一种高度恶性且罕见的软组织癌症，最常影响儿童。

主要特点

• 来源： SMS-CTR 细胞系是从一名胚胎性横纹肌肉瘤患者的肿瘤活检样本中建立的。

• 组织学分类： 它被归类为胚胎性横纹肌肉瘤（eRMS）。这是横纹肌肉瘤的主要亚型之一，与肺泡状亚型（aRMS）有所区别。这两个亚型之间一

• 个关键的分子差异在于是否存在基因融合。

• 融合状态： SMS-CTR 是一种融合阴性的细胞系。这意味着它不含有通常在更具侵袭性的肺泡状横纹肌肉瘤亚型中发现的 PAX-FOXO1 基因融合。这使其成为研究融合阴性 ERMS 的宝贵模型，因为这种亚型与融合阳性 aRMS 相比，可能具有不同的生物学特征和治疗反应。

• 遗传特征：

  • 它带有 HRAS 基因的突变（特别是 Q61K 突变）。这是一个重要特征，因为 RAS 通路突变是胚胎性横纹肌肉瘤常见的驱动因素。

  • 它还已知拥有p53 基因缺陷和其他染色体畸变。

研究中的应用

SMS-CTR 细胞系是横纹肌肉瘤，特别是融合阴性 ERMS 临床前研究的重要工具。其应用包括：

• 药物发现和筛选： 研究人员使用它来测试新的化疗药物和靶向疗法的有效性。由于其 HRAS 突变，它常用于研究 RAS-MAPK 信号通路的抑制剂。

• 研究肿瘤生物学： 它作为模型用于探究 ERMS 进展的分子机制，包括细胞增殖、分化和对治疗的耐药性。例如，研究已使用 SMS-CTR 细胞来了解对长春新碱等药物的耐药性是如何产生的。

• 遗传学和基因组学研究： 它用于分析横纹肌肉瘤的基因组图谱。研究人员可以研究其拷贝数变异、基因表达谱和染色质结构，以更好地理解这种疾病。

• 联合疗法： 该细胞系用于测试联合用药方案的疗效，例如将 MEK 抑制剂（如曲美替尼）与其他靶向药物联合使用。

The BioVector™ SMS-CTR cell line is a widely used human cancer cell line, specifically derived from a patient with embryonal rhabdomyosarcoma (ERMS). Rhabdomyosarcoma (RMS) is a highly aggressive and rare form of soft tissue cancer that most commonly affects children.

Key Characteristics

• Origin: The SMS-CTR cell line was established from a tumor biopsy of a patient with embryonal rhabdomyosarcoma.

• Histology: It is classified as an embryonal rhabdomyosarcoma (eRMS). This is one of the major subtypes of rhabdomyosarcoma, distinguished from the alveolar subtype (aRMS). A key molecular difference between these subtypes is the presence or absence of a gene fusion.

• Fusion Status: SMS-CTR is a fusion-negative cell line. This means it does not contain the characteristic PAX-FOXO1 gene fusions often found in the more aggressive alveolar rhabdomyosarcoma subtype. This makes it a valuable model for studying fusion-negative ERMS, which can have different biological characteristics and treatment responses compared to fusion-positive aRMS.

• Genetic Features:

  • It harbors a mutation in the HRAS gene (specifically, the Q61K mutation). This is a significant characteristic, as RAS pathway mutations are common drivers in embryonal rhabdomyosarcoma.

  • It is also known to have a defective p53 gene and other chromosomal alterations.

Applications in Research

The SMS-CTR cell line is a critical tool for preclinical research on rhabdomyosarcoma, particularly for fusion-negative ERMS. Its applications include:

• Drug Discovery and Screening: Researchers use it to test the effectiveness of new chemotherapeutic agents and targeted therapies. Because of its HRAS mutation, it is often used to study inhibitors of the RAS-MAPK signaling pathway.

• Studying Tumor Biology: It serves as a model to investigate the molecular mechanisms of ERMS progression, including cellular proliferation, differentiation, and resistance to therapy. For example, studies have used SMS-CTR cells to understand how resistance to drugs like vincristine develops.

• Genetics and Genomics Research: It is used to analyze the genomic landscape of rhabdomyosarcoma. Researchers can study its copy number variations, gene expression profiles, and chromatin structure to better understand the disease.

• Combination Therapies: The cell line is used to test the efficacy of combination drug regimens, such as combining MEK inhibitors (like trametinib) with other targeted agents.

BioVector NTCC质粒载体菌种细胞蛋白抗体基因保藏中心

BioVector NTCC Inc.

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E-mail: biovector@163.com

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