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货号： bs-4807R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4807R-AF350
- 英文名称
- Anti-ATXN3L/AF350
- 中文名称
- AF350标记的小脑脊髓共济失调蛋白3抗体
- 别 名
- ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 染色质和核信号 表观遗传学 泛素
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 41kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ATXN3L
- 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.
- 产品介绍
- 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.
Function:
Deubiquitinating enzyme that cleaves both Lys-48-linked and Lys-63-linked poly-ubiquitin chains (in vitro).
Subcellular Location:
Nucleus (By similarity).
Similarity:
Contains 1 Josephin domain.
Contains 2 UIM (ubiquitin-interacting motif) repeats.
Database links:
UniProtKB/Swiss-Prot: Q9H3M9.2
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.