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
毛片免费在线视频,1000午夜黄三级,日韩日韩日韩
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-NR0B1/FITC Conjugated antibody (bs-10434R-FITC)
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-FITC
英文名稱 Rabbit Anti-NR0B1/FITC Conjugated antibody
中文名稱 FITC標記的腎上腺發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.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: 少妇激情AV一区二区三区 | 欧美黄色一级片在线观看 | 欧美在线视频一区在线观看 | 无码三级香港经典三级在线视频 | 亚色九九九全国免费视频 | 日韩在线 | 色久综合一二码 | 精品熟女碰碰人人A久久 | 受被攻c哭高h视频在线观看 | 中文字幕一区二区日韩精品绯色 | 色婷婷国产精品一区二区 | 成人午夜激情视频 | 欧美精产国品一二三产品区别在哪 | 国内在线高清免费视频 | 日本黄色xxxxx | 日本一卡精品视频免费 | 日本老妇性视频 | 精品国产精品国产偷麻豆 | 亚洲v在线 | 在线欧美日韩 | 国产精品婷婷不卡久久久 | 噜噜色.com | 我要色综合色综合久久 | 4hu四虎影视入口 | 国内精品一区二区 | 视频分类国内精品 | 久久久无码一区二区三区 | 18videossex性欧美69 | 久久水蜜桃亚洲AV无码精品 | 国产美女久久 | 精品国内二区三区 | 亚洲免费视频播放 | 一级毛片免费一级 | 精品无码日韩一区二区三区不卡 | 极品少妇粉嫩小泬v片可看 99热婷婷 | 免费看18禁止观看黄网站 | wwxx在线观看免费 | 欧美Ⅴ永久无码精品毛片 | chinese45丰满成熟hd | 国产一区影院 | 日韩色综合 |