| 產品編號 | bs-12515R |
| 英文名稱 | Argininosuccinate Lyase Rabbit pAb |
| 中文名稱 | 琥珀酸裂解酶抗體 |
| 別 名 | Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1. |
| 研究領域 | 腫瘤 細胞生物 信號轉導 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應 | (predicted: Human,Mouse,Rat,Pig,Dog,Horse) |
| 產品應用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 52 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞漿 細胞外基質 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ASL: 301-400/464 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產品介紹 |
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. SWISS: P04424 Gene ID: 435 Database links: Entrez Gene: 435 Human Entrez Gene: 109900 Mouse Omim: 608310 Human SwissProt: P04424 Human SwissProt: Q91YI0 Mouse Unigene: 632015 Human Unigene: 23869 Mouse Unigene: 64591 Rat |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
