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
亚洲日韩久久精品无码AV | 国产精品自在拍在线播放 | 国产又粗又猛又爽又黄的视频免费软件 | www免费视频在线观看播放 | 91免费视频网站 | 精品国产福利一区二区在线 | YELLOW免费观看完整版直播 | 国产精品TV在线麻豆 | 免费看撕开奶罩揉吮奶头视频 | 欧美成人免费观看久久 | 成人做受120秒试看动态图 | 中文字幕无码一区二区免费 | 又大又粗进出白浆直流视频在线 | 扒开腿挺进湿润的花苞 | 人妻美妇疯狂迎合系列视频 | 波多野结衣AV全免费观 | 国产亚洲日韩在线播放人成 | 日韩无码高清福利在线看 | 欧美激情成a人在线观看 | 性妇WBBBB搡BBBB嗓小说 | 日韩精品福利视频一区二区三区 | 亚洲线精品一区二区三区八戒 | 女人18毛片a级毛片 久久精品国产亚洲AV蜜臀 | 女人被男人吃奶到高潮 | 国产又爽又黄无码无遮挡在线观点 | 黃色AV片三級三級三級免费看 | 亚洲色欲AV无码成人专区 | 亚洲AV无码乱码在线观看性色 | 亚洲AV无码李区亚洲A | 大肉大捧一进一出的视频 | jizz国产丝袜18老师美女 | 麻豆AV无码精品一区二区 | 成人免费精品网站在线观看影片 | 日韩一区二区在线观看视频 | 久久久久久精品免费自慰 | 国产精品一区二区AV日韩在线 | 免费A级毛片无码无遮挡 | 嫩B人妻精品一区二区三区 女人和拘做受全过程免费 日本亚洲一区二区三区 | 日韩人妻高清精品专区 | 少妇人妻偷人精品无码视频新浪 | 午夜男女爽爽爽免费播放 |