BD-PN1978

NR0B1 Rabbit Polyclonal Antibody

常规抗体

NR0B1 AHC DAX1

Nuclear receptor subfamily 0 group B member 1 (DSS-AHC critical region on the X chromosome protein 1) (Nuclear receptor DAX-1)

WB

Human,Rat,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.

190

P51843

https://www.uniprot.org/uniprotkb/P51843/entry

Q61066

http://www.uniprot.org/uniprot/Q61066

P70503

http://www.uniprot.org/uniprot/O54941P70503

Synthesized peptide derived from part region of human protein

NR0B1 Polyclonal Antibody detects endogenous levels of protein.

WB 1:500-2000 ELISA 1:5000-20000

51kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

Lung,

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 (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.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.