BioVector® UOK268 人遗传性平滑肌瘤病和肾细胞癌细胞系 / BioVector® UOK268 Human Hereditary Leiomyomatosis and Renal Cell Cancer Cell Line

通用定义 / General Definition:BioVector® UOK268 是一种源自人类遗传性平滑肌瘤病和肾细胞癌 (HLRCC) 的肾肿瘤细胞系。与著名的 UOK262 类似，UOK268 也是研究 延胡索酸水合酶 (Fumarate Hydratase, FH) 缺失性肾癌的关键生物模型。该细胞系源自一名 HLRCC 患者的原发性肾肿瘤，具有 FH 基因的纯合缺失或功能丧失性突变。FH 的缺失导致细胞内延胡索酸（Fumarate）浓度异常升高，进而诱导蛋白质琥珀酸化（Succination）和代谢重编程。UOK268 为科学家提供了比较不同患者来源、不同转化背景下 HLRCC 肿瘤生物学差异的宝贵资源。

BioVector® UOK268 is a renal tumor cell line derived from a patient with Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC). Similar to the well-known UOK262, UOK268 is a critical biological model for studying Fumarate Hydratase (FH)-deficient renal cell carcinoma. This cell line was established from the primary renal tumor of an HLRCC patient and features a homozygous deletion or loss-of-function mutation in the FH gene. The absence of FH leads to abnormally high levels of intracellular fumarate, which induces protein succination and metabolic reprogramming. UOK268 provides researchers with a valuable resource to compare biological variations across different patient-derived HLRCC models.

BioVector® UOK268 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® UOK268 人 HLRCC 肾细胞癌细胞

• 组织来源： 肾脏 (原发性肾癌)

• 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，呈多角形，贴壁牢固

2. 培养条件

• 基础培养基： BioVector® DMEM 培养基（高糖，含 4.5g/L 葡萄糖）。

• 血清添加： 10% BioVector® 优质胎牛血清 (FBS)。

• 关键添加剂： 建议添加 1mM 丙酮酸钠 (Sodium Pyruvate) 以支持其特殊的代谢需求。

• 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 85% 时进行传代。

3. 细胞应用

• 代谢机制研究： 研究延胡索酸积累如何通过抑制脯氨酰羟化酶 (PHDs) 导致 HIF-1$\alpha$ 的稳定性增强。

• 蛋白质组学： 鉴定由高浓度延胡索酸引起的异常蛋白质半胱氨酸琥珀酸化修饰。

• 精准医疗： 筛选能够针对性杀死 FH 缺失细胞的合成致死（Synthetic Lethality）药物。

4. 注意事项

• 营养耗竭： 由于 FH 缺失细胞极度依赖糖酵解，培养基中的葡萄糖消耗极快，建议缩短换液周期。

• 鉴定标准： 实验开始前应验证其 FH 蛋白缺失以及 2-琥珀酸半胱氨酸 (2-SC) 的高表达。

English Datasheet

1. General Product Information

• Product Name: BioVector® UOK268 Human HLRCC Renal Cell Cancer Cell Line

• Tissue Source: Kidney (Primary Renal Tumor)

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

• Growth Properties: Adherent

• Morphology: Epithelial-like, polygonal, and strongly adherent.

2. Culture Conditions

• Basal Medium: BioVector® DMEM (High Glucose, 4.5g/L glucose).

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

• Supplements: 1mM Sodium Pyruvate is recommended to support specialized metabolic requirements.

• Incubation: 37 degrees Celsius, 5% CO2.

• Subculturing: 1:2 to 1:4 ratio; subculture when cells reach 85% confluence.

3. Applications

• Metabolic Mechanisms: Investigating how fumarate accumulation enhances HIF-1$\alpha$ stability by inhibiting prolyl hydroxylases (PHDs).

• Proteomics: Identifying aberrant protein cysteine succination modifications caused by high intracellular fumarate concentrations.

• Precision Medicine: Screening for synthetic lethal drug candidates that selectively target FH-deficient cells.

4. Key Usage Notes

• Nutrient Depletion: FH-deficient cells are highly glycolytic and consume glucose rapidly; BioVector® recommends more frequent medium changes to prevent acidification and nutrient exhaustion.

• Characterization: Before initiating experiments, verify the absence of FH protein and the high expression of 2-succinyl cysteine (2-SC) via immunomarkers.

注意 / Note: BioVector® UOK268 应在生物安全二级 (BSL-2) 条件下操作。该细胞系对于研究 HLRCC 这种侵袭性极强的肾癌具有不可替代的作用，建议在实验中使用低代数细胞以保持其代谢特征。BioVector® UOK268 should be handled under Biosafety Level 2 (BSL-2) conditions. This cell line is indispensable for studying the highly aggressive HLRCC; BioVector® recommends using low-passage cells to preserve their unique metabolic profile.

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