BioVector® UOK262 人遗传性平滑肌瘤病和肾细胞癌细胞系 / BioVector® UOK262 Human Hereditary Leiomyomatosis and Renal Cell Cancer Cell Line

通用定义 / General Definition:BioVector® UOK262 是一种极其罕见且具有重要科研价值的人源遗传性平滑肌瘤病和肾细胞癌 (HLRCC) 细胞系。该细胞系源自一名患有 HLRCC 的患者的转移性腹膜后淋巴结。其核心特征是存在延胡索酸水合酶 (Fumarate Hydratase, FH) 基因的纯合失活突变。FH 的缺失导致细胞内延胡索酸积累，进而引发代谢重编程（Warburg 效应），使细胞高度依赖糖酵解。UOK262 是研究代谢异常诱发癌症（代谢肿瘤学）、FH 缺陷驱动的信号传导以及开发针对性代谢疗法的核心模型。

BioVector® UOK262 is a rare and highly significant human Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) cell line. Derived from a metastatic retroperitoneal lymph node of an HLRCC patient, its defining feature is the homozygous inactivation of the Fumarate Hydratase (FH) gene. The loss of FH leads to the accumulation of intracellular fumarate, triggering profound metabolic reprogramming (the Warburg effect) and making the cells highly dependent on glycolysis. UOK262 serves as a pivotal model for studying metabolic-driven oncogenesis (metabolic oncology), FH-deficiency signaling, and developing targeted metabolic therapies.

BioVector® UOK262 技术说明书 (Technical Datasheet)

中文版说明书 (Chinese Datasheet)

1. 产品基本信息

• 产品名称： BioVector® UOK262 人 HLRCC 肾细胞癌细胞

• 组织来源： 淋巴结 (转移自肾乳头状癌)

• 疾病背景： 遗传性平滑肌瘤病和肾细胞癌 (HLRCC)

• 生长特性： 贴壁生长

• 细胞形态： 上皮样，具有明显的异质性

2. 培养条件

• 基础培养基： BioVector® DMEM 培养基（高糖）。由于其代谢缺陷，必须确保葡萄糖供应充足。

• 血清添加： 10% BioVector® 优质胎牛血清 (FBS)。

• 关键添加剂： 某些研究建议补充丙酮酸钠 (Sodium Pyruvate)。

• 培养环境： 37 摄氏度，5% CO2

• 传代比例： 1:2 至 1:4；建议在细胞汇合度达到 80% 左右时传代。

3. 细胞应用

• 代谢肿瘤学： 研究 FH 缺失导致的克雷布斯循环（TCA 循环）中断及其对细胞能量代谢的影响。

• 信号通路： 探索延胡索酸积累诱导的假性缺氧通路（HIF-1$\alpha$ 稳定）和抗氧化应答通路（NRF2 活化）。

• 药物筛选： 开发靶向糖酵解途径或氧化磷酸化的新型抗癌药物。

4. 注意事项

• 代谢稳定性： 长期培养中应严密监测培养基 pH 值，因为高度活跃的糖酵解会导致乳酸迅速积累。

• 基因型鉴定： 确认 FH 表达缺失是使用该细胞系的前提，建议定期进行蛋白印迹 (Western Blot) 验证。

English Datasheet

1. General Product Information

• Product Name: BioVector® UOK262 Human HLRCC Renal Cell Cancer Cell Line

• Tissue Source: Lymph Node (Metastatic from papillary renal cell carcinoma)

• Disease Context: Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)

• Growth Properties: Adherent

• Morphology: Epithelial-like, exhibiting significant heterogeneity.

2. Culture Conditions

• Basal Medium: BioVector® DMEM (High Glucose). Due to metabolic defects, sufficient glucose supply is critical.

• Serum Supplement: 10% BioVector® Fetal Bovine Serum (FBS).

• Supplements: Sodium Pyruvate supplementation is often recommended.

• Incubation: 37 degrees Celsius, 5% CO2.

• Subculturing: 1:2 to 1:4 ratio; subculture when cells reach approximately 80% confluence.

3. Applications

• Metabolic Oncology: Investigating the disruption of the Krebs cycle (TCA cycle) caused by FH loss and its impact on cellular energy metabolism.

• Signaling Pathways: Exploring fumarate-induced pseudohypoxia (HIF-1$\alpha$ stabilization) and the antioxidant response (NRF2 activation).

• Drug Discovery: Screening for novel compounds that target glycolysis or exploit metabolic vulnerabilities in FH-deficient cells.

4. Key Usage Notes

• Metabolic Management: Monitor the pH of the culture medium closely, as aggressive glycolysis leads to rapid lactic acid accumulation.

• Genotype Validation: The absence of FH expression is the defining characteristic; BioVector® recommends periodic validation via Western Blot or enzymatic activity assays.

注意 / Note: BioVector® UOK262 应在生物安全二级 (BSL-2) 条件下操作。该细胞系对代谢环境极其敏感，建议使用经过预验证的 BioVector® 培养试剂盒以确保实验重现性。BioVector® UOK262 should be handled under Biosafety Level 2 (BSL-2) conditions. Given its extreme sensitivity to the metabolic environment, BioVector® recommends using pre-validated culture kits to ensure experimental reproducibility.

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