FITC标记的赖氨酸酮戊二酸还原酶抗体
产品名称： FITC标记的赖氨酸酮戊二酸还原酶抗体
英文名称： Anti-AASS/FITC
产品编号： HZ-11391R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-AASS/FITC Conjugated antibody
FITC标记的赖氨酸酮戊二酸还原酶抗体
| 产品编号 | bs-11391R-FITC |
| 英文名称 | Anti-AASS/FITC |
| 中文名称 | FITC标记的赖氨酸酮戊二酸还原酶抗体 |
| 别 名 | Alpha aminoadipic semialdehyde synthase mitochondrial; LKR/SDH; LKRSDH; LORSDH; Lysine ketoglutarate reductase; Saccharopine dehydrogenase; AASS_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 99kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human LKRSDH (878-926aa) |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: Alpha-aminoadipic semialdehyde synthase (AASS), also designated lysine ketoglutarate reductase (LKR) or saccharopine dehydrogenase (SDH), is a 926 amino acid protein that exists as a homodimer in the mitochondria. AASS acts as a bifunctional enzyme containing the lysine alpha-ketoglutarate reductase (LKR) and saccharopine dehydrogenase activities that catalyzes the first two steps in lysine degradation. It is widely expressed with highest expression in liver and transcription of the AASS gene is induced upon starvation. Mutations in the gene encoding AASS result in various forms familial hyperlysinemias (FH), autosomal recessive disorders characterized by hyperlysinemia, lysinuria, and variable saccharopinuria. However, no adverse mental or physical effects have been found in patients with hyperlysinemia. Function: Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. Subunit: Homodimer Subcellular Location: Mitochondrial Tissue Specificity: Expressed in all 16 tissues examined with highest expression in the liver. DISEASE: Defects in AASS are the cause of hyperlysinemia (HYPLYS) [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria. Similarity: In the N-terminal section; belongs to the AlaDH/PNT family. In the C-terminal section; belongs to the saccharopine dehydrogenase family. Database links: Entrez Gene: 10157 Human Omim: 605113 Human SwissProt: Q9UDR5 Human Unigene: 156738 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
α-氨基己二酸半醛合酶（AASS），又称赖氨酸酮戊二酸还原酶（LKR）或糖肽脱氢酶（SDH），是线粒体中同源二聚体存在的926个氨基酸。AASS作为一种双功能酶，含有赖氨酸-α-酮戊二酸还原酶（LKR）和糖蛋白脱氢酶活性，催化赖氨酸降解的前两个步骤。它在肝脏中广泛表达，AASS基因的转录在饥饿时被诱导。AASS基因的突变导致多种形式的家族性高赖氨酸血症（FH），常染色体隐性遗传疾病以高赖氨酸血症、赖氨酸尿和可变糖尿症为特征。然而，在高赖氨酸血症患者中没有发现不良的心理或物理效应。