FITC标记的AICAR甲酰基转移酶抗体
产品名称： FITC标记的AICAR甲酰基转移酶抗体
英文名称： Anti-ATIC/PURH/FITC
产品编号： HZ-12544R-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-ATIC/PURH/FITC Conjugated antibody
FITC标记的AICAR甲酰基转移酶抗体
| 英文名称 | Anti-ATIC/PURH/FITC |
| 中文名称 | FITC标记的AICAR甲酰基转移酶抗体 |
| 别 名 | AICAR transformylase; 5 aminoimidazole 4 carboxamide 1 beta D ribonucleotide transformylase/inosinicase; 5 aminoimidazole 4 carboxamide ribonucleotide formyltransferase; 5 aminoimidazole 4 carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase; 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase; AICAR; AICAR formyltransferase/IMP cyclohydrolase bifunctional enzyme; AICARFT; AICARFT/IMPCHASE; ATIC; Bifunctional purine biosynthesis protein PURH; FLJ93545; IMP cyclohydrolase; IMP synthase; IMP synthetase; IMPCHASE; Inosinicase; OK/SW-cl.86; Phosphoribosylaminoimidazolecarboxamide formyltransferase; Phosphoribosylaminoimidazolecarboxamide formyltransferase/IMP cyclohydrolase; PUR9_HUMAN; PURH. |
| 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 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, |
| 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 | 65kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ATIC/AICAR transformylase |
| 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: The bifunctional purine biosynthesis protein ATIC (also designated PURH) contains AICAR transformylase and IMP cyclohydrolase activities. AICAR (5-aminoimidazole-4-carboxamide ribonucleotide) transformylase catalyzes the second to last step in purine biosynthesis, playing an important role in the production of nucleotides and IMP. Defects in the ATIC transformylase gene can cause AICA-rebsuria, also designated AICA-ribosiduria, an inborn error in purine biosynthesis that is neurologically cataclysmic. Individuals with AICA-rebosuria accumulate AICA-riboside, also designated ZMP, and its derivatives in erythrocytes and fibroblasts. Patients also excrete very large amounts of AICA-riboside in the urine. Mental retardation, epilepsy, dysmorphic features and congenital blindness are all symptoms of this disease. Function: Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis. Subunit: Homodimer. DISEASE: Defects in ATIC are the cause of AICA-ribosuria [MIM:608688]; also known as AICA-ribosiduria. AICA-ribosuria is a neurologically devastating inborn error of purine biosynthesis. AICA-ribosuria patients excrete massive amounts of AICA-riboside in the urine and accumulate AICA-ribotide and its derivatives in erythrocytes and fibroblasts. AICA-ribosuria causes profound mental retardation, epilepsy, dysmorphic features and congenital blindness. Similarity: Belongs to the purH family. Database links: Entrez Gene: 471 Human Entrez Gene: 108147 Mouse Entrez Gene: 81643 Rat Entrez Gene: 396091 Chicken Omim: 601731 Human SwissProt: P31335 Chicken SwissProt: P31939 Human SwissProt: Q9CWJ9 Mouse SwissProt: O35567 Rat Unigene: 90280 Human Unigene: 38010 Mouse Unigene: 15114 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
双功能嘌呤生物合成蛋白质（也指定PURH）包含AICAR转化酶和IMP环水解酶活性。AICAR（5-氨基咪唑-4-羧基酰胺核糖核苷酸）转化酶催化嘌呤生物合成的第二步到最后一步，在核苷酸和IMPs的产生中起着重要的作用。转化酶基因缺陷可引起AICA排斥反应，也被命名为AICA核糖尿症。嘌呤生物合成中的先天性错误，神经病学上是灾难性的。AICA RebSur尿尿的个体积累了AICA核苷，也被命名为ZMP，其衍生物在红细胞和成纤维细胞中。患者还排出大量的AICA核苷在尿液中。智力低下、癫痫、畸形特征和先天性失明都是这种疾病的症状。