CLN5 抗体 (AA 61-120) (AbBy Fluor® 680)
-
- 抗原 See all CLN5 抗体
- CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))
-
抗原表位
- AA 61-120
-
适用
- 人
-
宿主
- 兔
-
克隆类型
- 多克隆
-
标记
- This CLN5 antibody is conjugated to AbBy Fluor® 680
-
应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 人
- 预测反应
- Mouse,Rat,Cow,Sheep,Pig,Horse,Rabbit
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human CLN5
- 亚型
- IgG
- Top Product
- Discover our top product CLN5 Primary Antibody
-
-
- 应用备注
-
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
-
- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
-
- 抗原
- CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))
- 别名
- CLN5 (CLN5 产品)
- 别名
- NCL antibody, A730075N08Rik antibody, CLN5, intracellular trafficking protein antibody, ceroid-lipofuscinosis, neuronal 5 antibody, CLN5 antibody, Cln5 antibody
- 背景
-
Synonyms: Ceroid lipofuscinosis neuronal 5, Ceroid-lipofuscinosis neuronal protein 5, CLN5, CLN5_HUMAN, NCL, Protein CLN5.
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.
-