FITC标记的小鼠抗棕榈酰蛋白硫酯酶1单克隆抗体上海沪震实业有限公司

性能参数

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
抗体货号: HZ-51262M-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/8/10 13:31:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
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产品详细描述

 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。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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