97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
国产精品夜夜夜一区二区三区尤,97永久免费视频,11www色com
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-phospho-Ataxin 1(Ser775)/PE-Cy5 Conjugated antibody (bs-12534R-PE-Cy5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12534R-PE-Cy5
英文名稱 Rabbit Anti-phospho-Ataxin 1(Ser775)/PE-Cy5 Conjugated antibody
中文名稱 PE-Cy5標記的磷酸化脊髓小腦失調癥蛋白1抗體
別    名 Ataxin 1 (phospho S776);p-Ataxin 1 (phospho S776); ATX1; ATXN1; SCA1; Ataxin 1; Ataxin-1; Ataxin1; Spinocerebellar ataxia type 1; ATX1_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產品類型 磷酸化抗體 
研究領域 細胞生物  神經生物學  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 87kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthesised phosphopeptide derived from human Ataxin 1 around the phosphorylation site of Ser776
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 background:
Ataxin-1, also designated spinocerebellar ataxia type 1 protein (Sca-1), is differentially expressed and localizes to both the cytoplasm and the nucleus. Mutations in Ataxin-1 are associated with the onset of the autosomal dominant neurodegenerative disorder spinocerebellar ataxia type 1 (SCA-1), which is characterized by progressive neuronal loss in the cerebellum, muscle wasting and ataxia. In Purkinje cells, where SCA-1 is predominantly observed, Ataxin-1 has been shown to directly associate with the Purkinje-enriched leucine-rich acidic nuclear protein (LANP) and the nuclear matrix-associated protein promyelocytic leukemia protein PML. In SCA-1, Ataxin-1 is mutated to encode a polyglutamine protein that forms nuclear aggregates, which interact significantly more strongly with LANP and contribute to the pathogenesis of SCA-1.

Function:
Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.

Subunit:
Homooligomer. Interacts with CIC (By similarity). Interacts with ANP32A, PQBP1, UBQLN4, ATXN1L, USP7 and ZNF804A. Directly interacts with RBPJ; this interaction is disrupted in the presence of Notch intracellular domain. Competes with ATXN1L for RBPJ-binding.

Subcellular Location:
Cytoplasm. Nucleus. Colocalizes with USP7 in the nucleus.

Tissue Specificity:
Widely expressed throughout the body.

Post-translational modifications:
Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract.
Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.

DISEASE:
Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) [MIM:164400]; also known as olivopontocerebellar atrophy I (OPCA I or OPCA1). 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 cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 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. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Similarity:
Belongs to the ATXN1 family.
Contains 1 AXH domain.

Database links:

Entrez Gene: 6310 Human

Entrez Gene: 20238 Mouse

Entrez Gene: 25049 Rat

Omim: 601556 Human

SwissProt: P54253 Human

SwissProt: P54254 Mouse

SwissProt: Q63540 Rat

Unigene: 434961 Human

Unigene: 342683 Mouse

Unigene: 342686 Mouse

Unigene: 88438 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 亚洲日韩爆乳中文字幕欧美 | 97人妻熟女成人免费视频 | ccyy草草影院地址入口 | 扒开双腿猛进入在线观看 | 欧美天天综合色影久久精品 | 久久艳片 | 午夜无码片在线观看影视 | 狠狠色狠狠色很很综合很久久 | 久久青草免费视频 | 久久久久无 | 爱福利在线视频 | 一区二区三区波多野结衣在线观看 | 18gay男同69亚洲帅男蓝宇 | 99久久久国产精品免费蜜臀 | 青青草91久久久久久久久 | 一区二区三区日本 | 日韩精品一区二区三区蜜臀 | 国产激情无码视频在线播放性色 | 成人综合色站 | 波多野结衣先锋在线 | 一区二区免费在线观看 | 国产精品夜间视频香蕉 | 黄色成人免费视频 | 97久久久久亚洲 | 波多野结衣无码免费视频 | 51精品国产人成在线观看 | 99国产精品国产免费观看 | 亚洲美女久久久 | 日本黄色xxxxx | 看黄色免费片 | 521av永久免费网 | 激情综合网俺也去 | 国产日韩欧美亚洲 | 水野优香在线亚洲一区 | 亚洲最大天堂网 | 夜夜调教禁脔欢爱h | 欧美专区在线 | 国产国语农村妇女偷人视频 | 亚洲精品无码久久久久AV老牛 | 免费看三片在线播放 | 国产精品视频www |