ITN1978
ITN1978
- Catalog: ITN1978
- Gene/Protein: NR0B1 AHC DAX1
- Product Description: Immunotag™ NR0B1 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
|
| Target Protein | NR0B1 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from part region of human protein |
| Specificity | NR0B1 Polyclonal Antibody detects endogenous levels of protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Gene Name | NR0B1 AHC DAX1 |
| Accession No. | P51843 Q61066 P70503 |
| Description | nuclear receptor subfamily 0 group B member 1(NR0B1) Homo sapiens This gene encodes a protein that contains a DNA-binding domain. The encoded protein acts as a dominant-negative regulator of transcription which is mediated by the retinoic acid receptor. This protein also functions as an anti-testis gene by acting antagonistically to Sry. Mutations in this gene result in both X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism. [provided by RefSeq, Jul 2008], |
| Protein Expression | Lung, |
| Subcellular Localization | nucleus,nucleoplasm,cytoplasm,membrane,polysomal ribosome, |
| Protein Function | disease:Defects in NR0B1 are the cause of X-linked adrenal hypoplasia congenital (AHC) [MIM:300200]. 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.,disease:XY individuals with a duplication of part of the short arm of the X chromosome and an intact SRY gene show dosage-sensitive sex reversal (DSS) [MIM:300018]. 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 DSS region are, therefore, not essential for testis development, but, when present in a double dose, interfere with testis formation.,domain:Homodimerization involved an interaction between amino and carboxy termini involving LXXLL motifs and steroid binding domain (AF-2 motif). Heterodimerizes with NR5A1/SF-1 and NROB2 through its N-terminal LXXLL motifs.,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.,similarity:Belongs to the nuclear hormone receptor family. NR0 subfamily.,subcellular location:Shuttles between the cytoplasm and nucleus. Homodimers exits in the cytoplasm and in the nucleus.,subunit:Homodimer. Interacts with NR5A1/SF-1, NR5A2, NR0B2 and with COPS2., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
| English_US |
|
| Danish |
|
| Dutch |
|
| English_UK |
|
| French |
|
| German |
|
| Spanish |
|
| Norwegian |
|
| Portuguese |
|
| Finnish |
|
| Swedish |
|
| Polish |
|