CLN8 抗体 (AA 201-286) (AbBy Fluor® 488)
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- 抗原 See all CLN8 抗体
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
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抗原表位
- AA 201-286
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适用
- 人, 大鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This CLN8 antibody is conjugated to AbBy Fluor® 488
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应用范围
- Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunofluorescence (Cultured Cells) (IF (cc))
- 交叉反应
- 人, 大鼠
- 预测反应
- Mouse,Dog,Pig,Horse,Rabbit
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human CLN8
- 亚型
- IgG
- Top Product
- Discover our top product CLN8 Primary Antibody
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- 应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
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- 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
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- 抗原
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
- 别名
- CLN8 (CLN8 产品)
- 别名
- mnd antibody, C8orf61 antibody, EPMR antibody, CLN8, transmembrane ER and ERGIC protein antibody, ceroid-lipofuscinosis, neuronal 8 antibody, CLN8 antibody, Cln8 antibody
- 背景
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Synonyms: Ceroid-lipofuscinosis, neuronal 8 epilepsy, progressive with mental retardation, Cln8, CLN8_HUMAN, EPMR, Protein CLN8, C8orf61
Background: CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC). Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR). Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which are associated with NCL.
- 基因ID
- 2055
- UniProt
- Q9UBY8
- 途径
- Regulation of Cell Size, Dicarboxylic Acid Transport
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