Anti-CLN5抗体Neuronal ceroid-lipofuscinose （NCL）, also designated Batten disease, comprises a group of recessively inherited,

产品编号 yb-11714R
英文名称 Anti-CLN5抗体
中文名称 神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体
别    名 Ceroid lipofuscinosis neuronal 5; Ceroid-lipofuscinosis neuronal protein 5; CLN5; CLN5_HUMAN; NCL; Protein CLN5.
Anti-CLN5抗体   
说 明 书 0.2ml  
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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.
分 子 量 37kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CLN5 (61-120aa)
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.

PubMed PubMed
产品介绍 background:
Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children. NCL is characterized by atrophy of the brain and an accumulation of lysosome derived fluorescent bodies found in many cells, especially neurons. Symptoms of NCL include a failure of psychomotor development, seizures, impaired vision and premature death. The eight genes/proteins associated with NCL are designated CLN1-CLN8. Mutations in six of these genes results in a distinct type of NCL-disease; the six genes/proteins are CLN1 (encoding PPT1, a protein thiolesterase), CLN2 (encodeing the serine protease TPP1), CLN3, CLN5, CLN6 and CLN8. A single base duplication mutation in dog and cow CLN5 has been shown to cause NCL.

Subcellular Location:
Lysosome.

Tissue Specificity:
Ubiquitous.

Post-translational modifications: