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  聯系我們
天堂8在线天堂资源BT,91手机在线视频观看,国产精品久久久久精品一级APP
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-NR0B1/BF350 Conjugated antibody (bs-10434R-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
產品編號 bs-10434R-BF350
英文名稱 Rabbit Anti-NR0B1/BF350 Conjugated antibody
中文名稱 BF350標記的腎上腺發(fā)育不全相關蛋白抗體
別    名 NR0B1 / Dax1; AHC; AHCH; AHX; DAX 1; DAX-1; DAX1; Dosage sensitive sex reversal; DSS; DSS AHC critical region on the X chromosome protein 1; DSS-AHC critical region on the X chromosome protein 1; GTD; HHG; Nr0b1; NR0B1_HUMAN; NROB1; Nuclear hormone receptor; Nuclear receptor 0B1; Nuclear receptor DAX 1; Nuclear receptor DAX-1; Nuclear receptor DAX1; Nuclear receptor subfamily 0 group B member 1; SRXY2.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  發(fā)育生物學  染色質和核信號  神經生物學  信號轉導  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
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 52kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NR0B1
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:
Adrenal hypoplasia congentia (AHC) is an X-linked disorder characterized by primary adrenal insufficiency. The disorder, which is lethal if untreated, results in adrenal insufficiency early in infancy and is characterized by low serum concentration of glucocorticoids, mineralcorticoids and androgens and failure to respond to ACTH. AHC has been mapped to chromosome Xp21 at the same or close to an X-linked locus involved in sex determination, DSS (for dosage-sensitive sex reversal). The gene corresponding to DSS and AHC (designated DAX-1 for DSS-AHC critical region on the X chromosome, gene 1) has been cloned and shown to be deleted in AHC deletion patients and mutated in AHC non-deletion patients. The carboxy terminal 250 amino acids of the DAX-1-encoded protein, DAX-1, exhibits approximately 50% continuous similarity to the ligand-binding domain of the members of the nuclear hormone receptor superfamily while the amino terminal domain contains a putative DNA-binding motif. DAX-1 binds to retinoic acid responsive elements and down regulates retinoic acid receptor-mediated transcriptional activation.

Function:
Orphan nuclear receptor. Component of a cascade required for the development of the hypothalamic-pituitary-adrenal-gonadal axis. Acts as a coregulatory protein that inhibits the transcriptional activity of other nuclear receptors through heterodimeric interactions. May also have a role in the development of the embryo and in the maintenance of embryonic stem cell pluripotency.

Subunit:
Homodimer. Interacts with NR5A1, NR5A2, NR0B2 and with COPS2.

Subcellular Location:
Nucleus. Cytoplasm. Shuttles between the cytoplasm and nucleus. Homodimers exits in the cytoplasm and in the nucleus.

DISEASE:
Defects in NR0B1 are the cause of X-linked adrenal hypoplasia congenital (XL-AHC) [MIM:300200]; also known as X-linked Addison disease (AHX). XL-AHC is a developmental disorder of the adrenal gland that results in profound hormonal deficiencies and is lethal if untreated. It is characterized by the absence of the permanent zone of the adrenal cortex and by a structural disorganization of the glands. Hypogonadotropic hypogonadism (HHG) is frequently associated with this disorder. HHG is a condition resulting from or characterized by abnormally decreased gonadal function, with retardation of growth and sexual development. Defects in NR0B1 are the cause of 46,XY sex reversal type 2 (SRXY2) [MIM:300018]. It is a condition characterized by male-to-female sex reversal in the presence of a normal 46,XY karyotype. Note=XY individuals with a duplication of part of the short arm of the X chromosome and an intact SRY gene develop as females. The single X chromosome in these individuals does not undergo X-chromosome inactivation; therefore, these individuals presumably carry 2 active copies of genes, including the NR0B1 gene, in the duplicated region. Individuals with deletion of this region develop as males. Genes within the dosage-sensitive sex reversal region are, therefore, not essential for testis development, but, when present in a double dose, interfere with testis formation.

Similarity:
Belongs to the nuclear hormone receptor family. NR0 subfamily.

Database links:

Entrez Gene: 450140 Chimpanzee

Entrez Gene: 190 Human

Entrez Gene: 11614 Mouse

Omim: 300473 Human

SwissProt: Q9BG97 Chimpanzee

SwissProt: P51843 Human

SwissProt: Q61066 Mouse

Unigene: 268490 Human

Unigene: 5180 Mouse



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         京公網安備110107000727號
国产精品久久久久久久久久免费 | 亚洲熟妇av一区二区三区色堂 | 91精品久久久久久久久久小网站 | 国产精品天天影视久久综合网 | 国精产品999一区二区三区有限 | 曰本a∨久久综合久久 | 日本老妇一级特黄aa大片 | 亚洲看片无码在线视频 | 久久久精品日本一区二区三区 | 学生被强奷到高潮喷水在线观看 | 一区二区三区日韩免费播放 | 亚洲国产精品一区二区动图 | 日韩精品在线观看免费 | 天天做天天添天天谢 | 日韩欧美视频一区 | 国产欧美精品一区二区色综合 | 好看的国产精彩视频 | 欧美极品欧美精品欧美视频 | 久久99国产精品一区二区 | 国产又爽又黄又爽又刺激 | 亚洲欧美一区二区久久 | 国产日韩亚洲VA无码自线免费看不卡 | 2012中文字幕一页免费 | 一本—道久久a久久精品蜜桃 | 又黄又爽又无遮挡在线观看免费 | 亚洲精品欧美精品日韩精品 | 黄色毛片视频免费 | 中文在线最新版天堂8 | 国产午夜亚洲精品国产 | 亚洲第一成年免费网站 | 国产精品99久久精品爆乳 | 免费又黄又硬又爽大片免费 | 中文字幕在线视频全部播放 | 精品日本亚洲一区二区三区 | 看亚洲A级一级毛片,亚洲AV片 | 精品国产麻豆免费人成网站 | 亚洲成人在线免费观看 | 国产精品秘 麻豆果冻传媒沈娜娜 | 亚洲国产高清国产拍精品 | 国产亚洲自拍一区 | 成年女人毛片免费播放视频m |