小脑脊髓共济失调蛋白3抗体
规格：1mg/1ml
英文名： ATXN3L
别名： ATX3L_HUMAN; ATXN3L; Machado-Joseph disease protein 1-like; MJDL; Putative ataxin-3-like protein.
分子量： 41kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ATXN3L
交叉反应：Human,
细胞定位：细胞膜
小脑脊髓共济失调蛋白3抗体产品介绍：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小脑脊髓共济失调蛋白3抗体 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:小脑脊髓共济失调蛋白3抗体 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.
小脑脊髓共济失调蛋白3抗体产品应用：WB=1:100-500 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学 染色质和核信号 表观遗传学 泛素
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid