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merck millipore,默克密理博,MAB5360,Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9
产品名称：Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9
产品型号：MAB5360
Detect Spinocerebellar Ataxia Type 3 using this Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 validated for use in ELISA, IC, IH, IP & WB.
merck millipore,默克密理博,MAB5360,Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 Mk, H, M, R WB, ELISA, ICC, IHC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB5360 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 Alternate Names - josephin
- Ataxin-3
背景信息 Spinocerebellar ataxia (SCA) is a genetic disease with multiple types, each of which could be considered a disease in its own right.
Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The first ataxia gene was identified in 1993 for a dominantly inherited type called “Spinocerebellar ataxia type 1" (SCA1). Subsequently, as additional dominant genes were found they were called SCA2, SCA3, etc. Usually, the "type" number of "SCA" refers to the order in which the gene was found. At this time, there are at least 29 different gene mutations which have been found.产品信息 格式 Ascites 控制 - Human SCA-3/MJD brain sections, NIH/3T3 lysate
演示 Ascites fluid containing no preservatives. 应用 应用 Detect Spinocerebellar Ataxia Type 3 using this Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 validated for use in ELISA, IC, IH, IP & WB. 主要应用 - Western Blotting
- ELISA
- Immunocytochemistry
- Immunohistochemistry
应用说明 Immunohistochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IH.
Immunoprecipitation:
A 1:500-1:5000 dilution of a previous lot was used in IP.
ELISA:
A 1:500-1:5000 dilution of a previous lot was used in ELISA.
Immunocytochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IC.
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Human ataxin-3 fragment from aa F112-L249 as a fusion protein 克隆 1H9 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 Ataxin-3. The epitope was mapped precisely at E214-L233. MAB5360 can be used to study wild type ataxin-3 and the mutant form with polyglutamine expansion found in patients affected with spinocerebellar ataxin type 3/Machado-Joseph disease (SCA3/MJD). In analysis of human tissues by Western blot, MAB5360 releaved several isoforms of ataxin-3 (presumably generated by alternative splicing, Trottier et al. 1998). The antibody detected polyglutamine aggregate (or nuclear inclusions) by IHC on SCA-3/MJD brain sections (Paulson et al. 1997). 同种型 IgG1 品种反应性 MonkeyHumanMouseRat Species Reactivity Note Human, monkey, rat and mouse 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_001024631.1
Entrez基因汇总 Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. 基因符号 - EC 3.4.22.-
- ATXN3
- SCA3
- JOS
- ATX3
- MJD1
- MJD
- AT3
纯化方法 Unpurified UniProt编号 - P54252
UniProt汇总 FUNCTION: SwissProt: P54252 # Interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription. Acts as a histone- binding protein that regulates transcription. Acts as a deubiquitinating enzyme.
SIZE: 376 amino acids; 43450 Da
SUBUNIT: Interacts with DNA repair proteins RAD23A and RAD23B.
SUBCELLULAR LOCATION: Nucleus matrix. Note=Predominantly nuclear, but not exclusively, inner nuclear matrix.
TISSUE SPECIFICITY: Ubiquitous.
DISEASE: SwissProt: P54252 # Defects in ATXN3 are the cause of spinocerebellar ataxia 3 (SCA3) [MIM:109150]; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of autosomal dominant cerebellar ataxias (ADCA). Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements. Spinocerebellar ataxias are caused by degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. Three clinical types are distinguished, according to the extent of extra-cerebellar signs: cerebellar ataxia with additional features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia, is known ADCA type I; cerebellar ataxia with retinal degeneration and pigmentary macular dystrophy is defined as ADCA type II; pure cerebellar ataxia without additional signs is classified as ADCA type III. SCA3 is clinically classified as ADCA type I. The molecular defect in SCA3 is the a CAG repeat expansion in ATX3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
SIMILARITY: SwissProt: P54252 ## Contains 1 Josephin domain. & Contains 3 UIM (ubiquitin-interacting motif) repeats.产品使用声明 质量保证 Evaluated by Western Blot on NIH/3T3 lysates.
Western Blot Analysis:
1:500 dilution of this antibody detected SPINOCEREBELLAR ATAXIA 3 on 10 µg of NIH/3T3 lysates.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at -20ºC from date of receipt. 包装信息 数量 100 µL
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