ATXN3L 抗体 (AA 251-355) (AbBy Fluor® 555)
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- 抗原 See all ATXN3L 抗体
- ATXN3L (Ataxin 3-Like (ATXN3L))
- 抗原表位
- AA 251-355
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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 ATXN3L antibody is conjugated to AbBy Fluor® 555
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 人
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human ATXN3L
- 亚型
- IgG
- Top Product
- Discover our top product ATXN3L 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
- ATXN3L (Ataxin 3-Like (ATXN3L))
- 别名
- ATXN3L (ATXN3L 产品)
- 别名
- MJDL antibody, ataxin 3 like antibody, ATXN3L antibody
- 背景
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Synonyms: ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.
Background: Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) , also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
- 基因ID
- 92552
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