Anti-Argininosuccinate Lyase/FITC Conjugated抗体
产品名称： Anti-Argininosuccinate Lyase/FITC Conjugated抗体
英文名称： Anti-Argininosuccinate Lyase/FITC
产品编号： YB--12515R-FITC
产品价格： null
产品产地： 中国/美国
品牌商标： Ybscience
更新时间： 2023-08-17T10:29:50
使用范围： 科研使用
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Anti-Argininosuccinate Lyase/FITC Conjugated抗体
| 产品编号 | YB-12515R-FITC |
| 英文名称 | Anti-Argininosuccinate Lyase/FITC |
| 中文名称 | FITC标记的琥珀酸裂解酶抗体 |
| 别 名 | Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1. |
| 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 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Horse, |
| 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 | 52kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase |
| 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: This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008] Function: Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 3/3. Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)succinate: step 1/1. Subcellular Location: Acetylation modifies enzyme activity in response to alterations of extracellular nutrient availability. Acetylation increased with trichostin A (TSA) or with nicotinamide (NAM). Glucose increases acetylation by about a factor of 3 with decreasing enzyme activity. Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity. DISEASE: Defects in ASL are the cause of arginosuccinicaciduria (ARGINSA) [MIM:207900]. Arginosuccinicaciduria is an autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness. Similarity: Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. Database links: Entrez Gene: 435 Human Entrez Gene: 109900 Mouse Entrez Gene: 59085 Rat Entrez Gene: 512771 Cow Omim: 608310 Human SwissProt: Q3SZJ0 Cow SwissProt: P04424 Human SwissProt: Q91YI0 Mouse SwissProt: P20673 Rat Unigene: 632015 Human Unigene: 23869 Mouse Unigene: 64591 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |