产品概述
| 产品名称（Product Name） | FH(7F1)Mouse Monoclonal Antibody |
| 描述（Description） | Mouse Monoclonal Antibody |
| 宿主（Host） | Mouse |
| 应用（Application） | WB,IHC-P,IF-P,IF-F,ICC/IF |
| 种属反应性（Reactivity） | Human,Mouse,Rat |
产品性能
| 偶联物（Conjugation） | Unconjugated |
| 修饰（Modification） | Unmodified |
| 同种型（Isotype） | IgG |
| 克隆（Clonality） | Monoclonal |
| 形式（Form） | Liquid |
| 存放说明（Storage） | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 储存溶液（Buffer） | PBS, pH 7.4, containing 0.5%BSA, 0.02% New type preservative N as Preservative and 50% Glycerol. |
| 纯化方式（Purification） | Affinity purification |
免疫原
| 基因名（Gene Name） | FH |
| 别名（Alternative Names） | Fumarate hydratase, mitochondrial (Fumarase) (EC 4.2.1.2) |
| 基因ID（Gene ID） | 2271 |
| 蛋白ID（SwissProt ID） | P07954 |
产品应用
| 稀释比（Dilution Ratio） | WB 1:3000, IF-P/IF-F/ICC/IF 1:200, IHC-P 1:50-300 |
| 蛋白分子量（Molecular Weight） | 50kDa |
研究背景
The protein encoded by this gene 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. It 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. [provided by RefSeq, Jul 2008],catalytic activity:(S)-malate = fumarate + H(2)O.,disease:Defects in FH are the cause of fumarase deficiency (FD) [MIM:606812]; also known as fumaricaciduria. FD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.,disease:Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].,disease:Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).,function:Also acts as a tumor suppressor.,miscellaneous:There are 2 substrate binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors.,pathway:Carbohydrate metabolism; tricarboxylic acid cycle.,PTM:Isoform Cytoplasmic is acetylated at position 2.,similarity:Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.,subunit:Homotetramer.,
研究领域
Citrate cycle (TCA cycle);Pathways in cancer;Renal cell carcinoma;
关键字： FH;FH(7F1)Mouse;Monoclonal;Antibody;一抗
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