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Fumarate Hydratase/Fumarase (BSB-151)
Fumarate Hydratase/Fumarase (BSB-151)
- 中文名称：
- Fumarate Hydratase/Fumarase (BSB-151)
- 英文名称：
- Fumarate Hydratase/Fumarase (BSB-151)
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA59370
- 规格：
- 5 Slides|3 mL (RTU)|0.1 mL (Concentrate)|7 mL (RTU)|0.5 mL (Concentrate)
- 保存建议：
- Store at 2-8 degree C (Control Slides: Store at 20-25 degree C)
- 货期：
- 6-8周
- 来源宿主：
- Mouse
- 反应种属：
- Human, Mouse, Rat
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Monoclonal
同型:IgG1
克隆号:[BSB-151]
特异性:N/A
纯度:N/A
形式：Paraffin, Frozen
Anti-fumarate hydratase is a mouse monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH7.5, containing BSA and sodium azide as a preservative.
浓度：N/A
- 说明书：
Fumarate hydratase (FH), or fumarase, is encoded by the FH gene, which is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, where it catalyzes the formation of L-malate from fumarate. Mutations in the FH gene can cause FH deficiency and lead to progressive encephalopathy. It was discovered that succinate dehydrogenase and FH are tumour suppressors and they are associated with metabolic dysfunction and tumorigenesis, providing biochemical evidence to explain enhanced glycolysis in tumours.
Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) is an autosomal dominant heritable syndrome with predisposition to development of Renal Cell Carcinoma and Smooth Muscle Tumors of the skin and uterus. Cells of individuals with HLRCC had lower fumarate hydratase antibody activity than cells from normal controls, making fumarate hydratase antibody activity testing a useful method for identification and screening. Loss-of-function mutations of FH predisposes individuals to the autosomal dominant syndrome of Multiple Cutaneous and Uterine Leiomyomatosis (MCUL). Biallelic inactivation/mutations of FH are seen in 85% of Hereditary Leiomyomatosis and Renal Cell Carcinoma cases, 100% of Renal Cell Carcinoma with germline FH mutations, 19% of Papillary Renal Cell Carcinoma (Type II) have FH deficiency and 90% of FH deficiency RCC have FH mutations, 1% of unselected Leiomyomas, 2.6% of Leiomyomas in patients < 40 years old and 37- 52% of Leiomyoma with bizarre nuclei.
