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
国产一级一片免费播放i,国产91精品系列在线观看
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-phospho-Ataxin 1(Ser775)/BF350 Conjugated antibody (bs-12534R-BF350)
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-BF350
英文名稱 Rabbit Anti-phospho-Ataxin 1(Ser775)/BF350 Conjugated antibody
中文名稱 BF350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 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
免费一级欧美片片线观看 | 日韩精品一区二区三区色欲AV | 亚洲AV无码乱码国产麻豆穿越 | 夜里十大禁用短视频软件 | 欧美freesex黑人又粗又大 | 丝袜自慰一区二区三区四区 | 久久国产V一级毛多内射 | 波多野结喷水最猛一部352 | 国产亚洲精品中文带字幕21页 | 日本欧美一区二区三区片 | 久久九九日本韩国精品 | 免费视频国产在线观看 | 天天做天天添天天谢 | 精品91自产拍在线观看二区 | 被伴郎的内捧猛烈进出H视频 | 一级毛片在线看在线播放 | 永久免费不卡在线观看黄网站 | 91久久精品无码一级毛片 | 国产又大又硬又粗 | 日韩免费精品视频 | 一本大道香蕉在线资源 | 91色老久久精品偷偷蜜臀 | 一级特黄录像视频免费 | 免费一级欧美片片线观看 | 国产精品一区二区久久不卡 | 啊灬啊灬啊灬快灬高潮了听书 | 人妻中文字幕乱人伦在线 | 亚洲无码总合另类中文字幕 | 日日噜噜噜夜夜爽爽狠狠 | 蘑菇视频在线观看MV看片 | 算你色永久免费视频播放 | 丰满爆乳肉感一区二区三区 | 4399视频免费观看大全 | 国产人成高清在线视频99 | 边做饭边被躁在线播放 | 久爱成欢视频在线观看 | 一本大道香蕉青青久久 | 91性高湖久久久久久久久 | 亚洲AV综合色区无码三区30p | 国产精品伦一区二区三级视频 | 国产精品久久久久久久久久影院 |