货号 |
bsm-33167M-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Mouse Anti-Fumarase/FH antibody |
中文名称 |
Mouse Anti-Fumarase/FH antibody |
研究领域 |
肿瘤,细胞生物,免疫学,转录调节因子,线抗体, |
英文别名 |
FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata. |
反应物种(已验证) |
Human,Rat |
反应物种(预测) |
Mouse |
产品应用(已验证) |
IHC |
产品应用(可推荐) |
IF |
推荐稀释比例 |
IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500, |
克隆类型 |
单克隆 |
克隆号 |
4C12 |
抗体来源 |
Mouse |
理论分子量 |
51 |
细胞定位 |
细胞浆 |
性状 |
Liquid |
免疫原 |
Recombinant human Fumarase/FH Protein |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein G |
SUBCELLULAR |
Cytoplasm, Mitochondrion |
Tissue |
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. |
SIMILARITY |
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. |
SUBUNIT |
Homotetramer. |
Function |
Also acts as a tumor suppressor. |
DISEASE |
Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, |
SWISS |
P07954 |
Gene ID |
2271 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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] |