FITC标记的丙酮酸羧化酶抗体
产品名称： FITC标记的丙酮酸羧化酶抗体
英文名称： Anti-PCB/FITC
产品编号： HZ-5000R-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： IF=1:50-200
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Rabbit Anti-PCB/FITC Conjugated antibody
FITC标记的丙酮酸羧化酶抗体
| 英文名称 | Anti-PCB/FITC |
| 中文名称 | FITC标记的丙酮酸羧化酶抗体 |
| 别 名 | mitochondrial; OTTHUMP00000235155; OTTHUMP00000235156; PC; PCB; Pcx; PYC_HUMAN; Pyruvate carboxylase; Pyruvate carboxylase mitochondrial; Pyruvic carboxylase. |
| 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 免疫学 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, Cow, Horse, Rabbit, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 127kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PCB |
| 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: PCB (Pyruvate carboxylase) requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. PCB is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency. Alternatively spliced transcript variants with different 5' UTRs, but encoding the same protein, have been found for this gene. Function: Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. Subunit: Homotetramer. Subcellular Location: Mitochondrion matrix. DISEASE: Pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]: Leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Contains 1 ATP-grasp domain. Contains 1 biotin carboxylation domain. Contains 1 biotinyl-binding domain. Contains 1 carboxyltransferase domain. Database links: UniProtKB/Swiss-Prot: P11498.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
PCB（Pyruvate carboxylase）需要生物素和ATP催化丙酮酸羧基化为草酰乙酸。活性酶是位于四面体中的同源四聚体，其位于线粒体基质中。PCB参与糖异生、脂肪生成、胰岛素分泌和神经递质谷氨酸的合成。该基因的突变与丙酮酸羧化酶缺乏有关。另一种剪接转录变体具有不同的5’UTR，但编码相同的蛋白质，已经发现该基因。