FITC标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体
产品名称： FITC标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体
英文名称： Anti-PPT1/FITC
产品编号： HZ-51262M-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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Mouse Anti-PPT1/FITC Conjugated antibody
FITC标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体
| 英文名称 | Anti-PPT1/FITC |
| 中文名称 | FITC标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体 |
| 别 名 | CLN1; INCL; Palmitoyl protein hydrolase 1; Palmitoyl protein thioesterase 1; Palmitoyl-protein hydrolase 1; Palmitoyl-protein thioesterase 1; PPT; PPT-1; PPT1; PPT1_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 信号转导 |
| 抗体来源 | Mouse |
| 克隆类型 | Monoclonal |
| 克 隆 号 | 10G4 |
| 交叉反应 | |
| 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 | 31kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 2mg/1ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PPT1 |
| 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 protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008] Function: Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons (PubMed:8816748) Subcellular Location: Lysosome. DISEASE: Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1) [MIM:256730]. A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, and adult onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). Similarity: Belongs to the palmitoyl-protein thioesterase family. Database links: Entrez Gene: 281421 Cow Entrez Gene: 5538 Human Entrez Gene: 19063 Mouse Entrez Gene: 29411 Rat Omim: 600722 Human SwissProt: P45478 Cow SwissProt: P50897 Human SwissProt: O88531 Mouse SwissProt: P45479 Rat Unigene: 3873 Human Unigene: 277719 Mouse Unigene: 1574 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
由该基因编码的蛋白质是一种小的糖蛋白，参与溶酶体降解过程中脂质修饰蛋白的分解代谢。编码的酶从半胱氨酸残基中除去硫酯连接的脂肪酰基，如棕榈酸。该基因的缺陷是婴儿神经元蜡样质脂褐素沉积症1（CLN1，或CYL）和神经元蜡样脂褐质沉积症4（CLN4）的一个原因。已经发现了两个编码不同亚型的转录变体，该基因由RefSeq提供，DEC 2008。