BioVector? UOK262 人遺傳性平滑肌瘤病和腎細胞癌細胞系 / BioVector? UOK262 Human Hereditary Leiomyomatosis and Renal Cell Cancer Cell Line

通用定義 / General Definition:BioVector? UOK262 是一種極其罕見且具有重要科研價值的人源遺傳性平滑肌瘤病和腎細胞癌 (HLRCC) 細胞系。該細胞系源自一名患有 HLRCC 的患者的轉移性腹膜后淋巴結。其核心特征是存在延胡索酸水合酶 (Fumarate Hydratase, FH) 基因的純合失活突變。FH 的缺失導致細胞內延胡索酸積累，進而引發(fā)代謝重編程（Warburg 效應），使細胞高度依賴糖酵解。UOK262 是研究代謝異常誘發(fā)癌癥（代謝腫瘤學）、FH 缺陷驅動的信號傳導以及開發(fā)針對性代謝療法的核心模型。

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 技術說明書 (Technical Datasheet)

中文版說明書 (Chinese Datasheet)

1. 產品基本信息

• 產品名稱： BioVector? UOK262 人 HLRCC 腎細胞癌細胞

• 組織來源： 淋巴結 (轉移自腎乳頭狀癌)

• 疾病背景： 遺傳性平滑肌瘤病和腎細胞癌 (HLRCC)

• 生長特性： 貼壁生長

• 細胞形態(tài)： 上皮樣，具有明顯的異質性

2. 培養(yǎng)條件

• 基礎培養(yǎng)基： BioVector? DMEM 培養(yǎng)基（高糖）。由于其代謝缺陷，必須確保葡萄糖供應充足。

• 血清添加： 10% BioVector? 優(yōu)質胎牛血清 (FBS)。

• 關鍵添加劑： 某些研究建議補充丙酮酸鈉 (Sodium Pyruvate)。

• 培養(yǎng)環(huán)境： 37 攝氏度，5% CO2

• 傳代比例： 1:2 至 1:4；建議在細胞匯合度達到 80% 左右時傳代。

3. 細胞應用

• 代謝腫瘤學： 研究 FH 缺失導致的克雷布斯循環(huán)（TCA 循環(huán)）中斷及其對細胞能量代謝的影響。

• 信號通路： 探索延胡索酸積累誘導的假性缺氧通路（HIF-1$\alpha$ 穩(wěn)定）和抗氧化應答通路（NRF2 活化）。

• 藥物篩選： 開發(fā)靶向糖酵解途徑或氧化磷酸化的新型抗癌藥物。

4. 注意事項

• 代謝穩(wěn)定性： 長期培養(yǎng)中應嚴密監(jiān)測培養(yǎng)基 pH 值，因為高度活躍的糖酵解會導致乳酸迅速積累。

• 基因型鑒定： 確認 FH 表達缺失是使用該細胞系的前提，建議定期進行蛋白印跡 (Western Blot) 驗證。

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 應在生物安全二級 (BSL-2) 條件下操作。該細胞系對代謝環(huán)境極其敏感，建議使用經過預驗證的 BioVector? 培養(yǎng)試劑盒以確保實驗重現(xiàn)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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