Item no. |
18-003-44442 |
Manufacturer |
GENWAY
|
Amount |
0.1 mg |
Category |
|
Type |
Antibody |
Applications |
WB, IHC |
Specific against |
other |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
GWB-CDF2AB |
Similar products |
18-003-44442 |
Available |
|
Genway ID: |
GWB-CDF2AB |
Antigen Specificity: |
Polyclonal antibody produced in rabbits immunized with a synthetic peptide corresponding to a region of Human NR0B1. WB: Antibody |
Dilution: |
2. 5ug/mlIHC: 4-8ug/ml |
Handling: |
Add 100ul of distilled water to this antibody before use. |
Immunogen: |
TSSKQTHVAPAAPEARPGGAWWDRSYFAQRPGGKEALPGGRATALLYRCC |
ELISA Titre: |
1:312500 |
Appearance: |
Lyophilized powder |
Note: |
Suggested starting concentrations are provided. Optimal dilutions should be determined by end-user. Differences in calculated versus apparent molecular weight may be due to post-translational modifications or protein hydrophobicity. NR0B1 is a protein that contains a DNA-binding domain. The 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 |
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 (By similarity). |
Subunit: |
Homodimer. Interacts with NR5A1/SF-1 NR5A2 NR0B2 and with COPS2. |
Subcellular Location: |
Nucleus. Cytoplasm. Note=Shuttles between the cytoplasm and nucleus. Homodimers exits in the cytoplasm and in the nucleus. |
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. |
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. |
Similarity: |
Belongs to the nuclear hormone receptor family. NR0 subfamily. Summary: This protein is 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. Mantovani. G. . (2005) Fertil. Steril. 84 (5). 1542-1544. |
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