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神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体

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产物名称: 神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体
产物型号: CLN3
产物展商: 单克隆抗体/多克隆抗体
产物文档: 无相关文档

简单介绍

神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体应用于IHC、WB、 IF、IP、ELISA等科研实验,按理化性质和生物学功能IgM、IgG、IgA、IgE、IgD五类。按抗体的来源,可将其分为天然抗体和**抗体。神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体生产每个流程都执行严格的检测标准,保证蛋白抗原产物质量,质量稳定,实验效果明显。


神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体  的详细介绍

神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体

规格:1尘驳/1尘濒

英文名: CLN3

别名: Batten disease protein; Battenin; BTS; Ceroid lipofuscinosis neuronal 3; Ceroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease); Ceroid lipofuscinosis neuronal 3 juvenile; CLN 3; M

分子量: 48kDa

储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型:笔辞濒测肠濒辞苍补濒

亚型:滨驳骋

纯化方法:affinity purified by Protein A

**原:KLH conjugated synthetic peptide derived from human CLN3

交叉反应:Human, Mouse, Rat, Cow, Horse, Rabbit, Monkey, macaque

细胞定位:细胞浆 细胞膜

神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体产物介绍:background: This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. Function: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Subunit: Interacts with DCTN1 and KIF3A. Interacts with RAB7A and RILP. Subcellular Location: Lysosome membrane; Multi-pass membrane protein. Late endosome Post-translational modifications: Highly glycosylated. Farnesylation is important for trafficking to lysosomes. DISEASE: Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. A form of neuronal ceroid lipofuscinosis. 神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体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 hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. 神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3. Similarity: Belongs to the battenin family. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

神经细胞蜡样质脂褐质沉积病蛋白颁尝狈3抗体品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究领域:细胞生物  神经生物学  

储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源: Rabbit

外观: Lyophilized or Liquid


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