亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

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(lián)系我們
久久精品黄AA片一区二区三区,亚洲国产精品久久精品成人网站
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-phospho-Ataxin 1(Ser775)/Biotin Conjugated antibody (bs-12534R-Bio)
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(chǎn)品編號 bs-12534R-Bio
英文名稱 Rabbit Anti-phospho-Ataxin 1(Ser775)/Biotin Conjugated antibody
中文名稱 生物素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(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產(chǎn)品類型 磷酸化抗體 
研究領域 細胞生物  神經(jīng)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(chǎn)品應用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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(chǎn)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.a6308.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(yī)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(wǎng)安備110107000727號
国产亚洲精品无码专区不卡 | 免费AV岛国大片在线观看 | 国产欧美日韩高清在线不卡 | 欧美日韩一区二区三区自拍 | 久久精品国产亚洲A片高清不卡 | 最近中文字幕在线看免费完整版 | 国产精品自慰在线观看 | 在线观看免费人成视频色 | 国产精品看高国产精品不卡 | 美女无遮挡无打码内射裸身视频 | 国产美女一级做a爱视频 | 丰满少妇又爽又紧又丰满在线观看 | 国产av中文字幕在线观看 | 国产精品一区二区久久不卡 | 日本亚洲一区二区三区 | 男男H黄动漫啪啪无遮挡网站 | 亚欧精品一区二区三区四区 | 亚洲一区爱区精品无码 | 久久久久久久岛国免费观看 | 一本一道久久a久久综合蜜桃 | 黄色视频免费在线观看 | 亚洲色欲色欲WWW成人网麻豆 | 国产嫖妓一区二区三区无码 | 欧美日韩永久久一区二区三区 | 无码人妻免费一区二区三区 | 50岁老熟女露脸25分钟91 | 91麻豆精品国产一级 | 免费一级毛片在线观看 | 无码午夜福利免费区久久 | 欧美成人se01短视频在线看 | 欧美极品欧美精品欧美视频 | WWW国产成人免费观看视频 | 天天躁日日躁狠狠躁欧美 | 精品国产第一福利网站 | 日本又黄又裸一级大黄裸片 | 91精品孕妇系列 | 国产大爆乳大爆乳在线播放 | 数学老师扒开腿让我爽快 | 亚洲综合另类欧美久久久久精品 | 台湾一级毛片永久免费 | 2018国产大陆天天弄 |