FITC标记的氨基甲酸乙酰转移酶抗体
产品名称： FITC标记的氨基甲酸乙酰转移酶抗体
英文名称： Anti-NAGS/FITC
产品编号： HZ-19004R-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-NAGS/FITC Conjugated antibody
FITC标记的氨基甲酸乙酰转移酶抗体
| 英文名称 | Anti-NAGS/FITC |
| 中文名称 | FITC标记的氨基甲酸乙酰转移酶抗体 |
| 别 名 | 1700120E20Rik; AGAS; AI415708; Amino-acid acetyltransferase; ARGA; EC 2.3.1.1; MGC133025; NAGS_HUMAN; N-acetylglutamate synthase; N-acetylglutamate synthase, mitochondrial; RP23-398F7.13. |
| 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 G蛋白信号 |
| 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 | 56kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human NAGS |
| 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 N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia. [provided by RefSeq, Jul 2008] Function: Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue. Post-translational modifications: Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated DISEASE: N-acetylglutamate synthase deficiency (NAGSD) [MIM:237310]: Rare autosomal recessively inherited metabolic disorder leading to severe neonatal or late-onset hyperammonemia without increased excretion of orotic acid. Clinical symptoms are somnolence, tachypnea, feeding difficulties, a severe neurologic presentation characterized by uncontrollable movements, developmental delay, visual impairment, failure to thrive and hyperammonemia precipitated by the introduction of high-protein diet or febrile illness. Similarity: Belongs to the acetyltransferase family. Contains 1 N-acetyltransferase domain. Database links: Entrez Gene: 162417 Human Entrez Gene: 490943 Dog Entrez Gene: 217214 Mouse SwissProt: Q8N159 Human SwissProt: Q8R4H7 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
N-乙酰谷氨酸合成酶基因编码一种线粒体酶，该酶催化由谷氨酸和乙酰辅酶A生成N-乙酰谷氨酸(NAG)。该基因可通过改变NAG的可用性和由此引起的CPSI活性来调节尿素生成。N-乙酰谷氨酸合成酶的缺乏与高氨血症有关。[ RefSeq，JUL 2008 ]