CLN6 抗体 (AA 101-200) (AbBy Fluor® 488)
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- 抗原 See all CLN6 抗体
- CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))
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抗原表位
- AA 101-200
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适用
- 人
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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 CLN6 antibody is conjugated to AbBy Fluor® 488
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 预测反应
- Human,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 CLN6
- 亚型
- IgG
- Top Product
- Discover our top product CLN6 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
- CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))
- 别名
- CLN6 (CLN6 产品)
- 别名
- 1810065L06Rik antibody, AW743417 antibody, D9Bwg1455e antibody, nclf antibody, CLN4A antibody, HsT18960 antibody, cln6 antibody, zgc:103565 antibody, ceroid-lipofuscinosis, neuronal 6 antibody, CLN6, transmembrane ER protein antibody, CLN6, transmembrane ER protein S homeolog antibody, ceroid-lipofuscinosis, neuronal 6, late infantile, variant antibody, CLN6, transmembrane ER protein a antibody, Cln6 antibody, CLN6 antibody, cln6.S antibody, cln6a antibody
- 背景
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Synonyms: Ceroid lipofuscinosis, neuronal 6, late infantile, variant, CLN6 protein, FLJ20561, Nclf, Protein CLN6, CLN6_HUMAN.
Background: CLN6, a 311 amino acid protein, has seven predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness, part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.
- 基因ID
- 54982
- UniProt
- Q9NWW5
- 途径
- Glycosaminoglycan Metabolic Process
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