Fumarate Hydratase (FH) is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. Fumarate Hydratase is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. Individuals with hemizygous germline fumarate hydratase (FH) mutations are predisposed to renal cancer. These tumors predominantly exhibit functional inactivation of the remaining wild-type allele, implicating FH inactivation as a tumor-promoting event.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
100 μg | ¥ 3,170 | 5日内发货 |
生物活性 | Measured by its ability to transform 1umole of Fumarate to L-malate per minute at pH 7.5 at 37°C. Specific activity is > 25 unit/mg. |
产品描述 | Fumarate Hydratase (FH) is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. Fumarate Hydratase is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. Individuals with hemizygous germline fumarate hydratase (FH) mutations are predisposed to renal cancer. These tumors predominantly exhibit functional inactivation of the remaining wild-type allele, implicating FH inactivation as a tumor-promoting event. |
种属 | Human |
表达系统 | E. coli |
标签 | His |
蛋白编号 | P07954-1 |
别名 | fumarate hydratase, MCL, LRCC, MCUL1, FMRD, HLRCC |
蛋白构建 | A DNA sequence encoding the mature form of human FH (P07954-1) (Ala 45-Lys 510) was fused with a polyhistidine tag at the C-terminus and an initial Met at the N-terminus. |
蛋白纯度 | > 85 % as determined by SDS-PAGE |
分子量 | 52 kDa (predicted) |
内毒素 | Please contact us for more information. |
缓冲液 | Lyophilized from sterile 10mM Tris, 5mM EDTA, 1mM DTT, pH 7.5. Please contact us for any concerns or special requirements. Normally 5 % - 8 % trehalose, mannitol and 0. 01% Tween 80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA. |
复溶方法 | A hardcopy of datasheet with reconstitution instructions is sent along with the products. Please refer to it for detailed information. |
存储 |
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles. |
运输方式 |
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise. |
研究背景 | Fumarate Hydratase (FH) is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. Fumarate Hydratase is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. Individuals with hemizygous germline fumarate hydratase (FH) mutations are predisposed to renal cancer. These tumors predominantly exhibit functional inactivation of the remaining wild-type allele, implicating FH inactivation as a tumor-promoting event. |
bottom
您可能有的问题的答案可以在重组蛋白操作手册中找到
FH/Fumarate Hydratase Protein, Human, Recombinant (His) fumarate hydratase MCL LRCC MCUL 1 MCUL1 FMRD MCUL-1 HLRCC recombinant recombinant-proteins proteins protein