Full Product Name
NR0B1, NT (Nuclear Receptor Subfamily 0 Group B Member 1, Nuclear Receptor DAX-1, DSS-AHC Critical Region On The X Chromosome Protein 1, DAX1, AHC, DAX1)
Product Synonym Names
Anti -NR0B1, NT (Nuclear Receptor Subfamily 0 Group B Member 1, Nuclear Receptor DAX-1, DSS-AHC Critical Region On The X Chromosome Protein 1, DAX1, AHC, DAX1)
Product Gene Name
anti-NR0B1 antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: X; NC_000023.10 (30322539..30327495, complement). Location: Xp21.3
3D Structure
ModBase 3D Structure for P51843
Specificity
Recognizes human NROB1.
Purity/Purification
Affinity Purified
Purified by immunoaffinity chromatography.
Form/Format
Supplied as a liquid in PBS, 0.09% sodium azide.
Immunogen
Synthetic peptide selected from the N-terminal region of human NROB1 (KLH).
Preparation and Storage
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Other Notes
Small volumes of anti-NR0B1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-NR0B1 antibody
NROB1 contains a DNA-binding domain and 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 the gene for NROB1 result in both X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism.
Product Categories/Family for anti-NR0B1 antibody
Antibodies; Abs to Receptors
Applications Tested/Suitable for anti-NR0B1 antibody
ELISA (EL/EIA), Western Blot (WB)
Application Notes for anti-NR0B1 antibody
Suitable for use in ELISA and Western Blot.
Dilution: ELISA: 1:1,000
Western Blot: 1:50-1:100
NCBI/Uniprot data below describe general gene information for NR0B1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000466.2
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NCBI GenBank Nucleotide #
NM_000475.4
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UniProt Primary Accession #
P51843
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UniProt Secondary Accession #
Q96F69[Other Products]
UniProt Related Accession #
P51843[Other Products]
Molecular Weight
51,718 Da[Similar Products]
NCBI Official Full Name
nuclear receptor subfamily 0 group B member 1
NCBI Official Synonym Full Names
nuclear receptor subfamily 0, group B, member 1
NCBI Official Symbol
NR0B1??[Similar Products]
NCBI Official Synonym Symbols
AHC; AHX; DSS; GTD; HHG; AHCH; DAX1; DAX-1; NROB1; SRXY2
??[Similar Products]
NCBI Protein Information
nuclear receptor subfamily 0 group B member 1; nuclear receptor DAX1; nuclear receptor DAX-1; nuclear hormone receptor; DSS-AHC critical region on the X chromosome protein 1
UniProt Protein Name
Nuclear receptor subfamily 0 group B member 1
UniProt Synonym Protein Names
DSS-AHC critical region on the X chromosome protein 1; Nuclear receptor DAX-1
Protein Family
Nuclear receptor subfamily
UniProt Gene Name
NR0B1??[Similar Products]
UniProt Synonym Gene Names
AHC; DAX1??[Similar Products]
UniProt Entry Name
NR0B1_HUMAN
NCBI Summary for NR0B1
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]
UniProt Comments for NR0B1
NR0B1: 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. Homodimerizes with STF-1, NR5A2, NR0B2 and with COPS2. Shuttles between the cytoplasm and nucleus. Homodimers exits in the cytoplasm and in the nucleus. Homodimerization involved an interaction between amino and carboxy termini involving LXXLL motifs and steroid binding domain (AF-2 motif). Heterodimerizes with STF-1 and NROB2 through its N-terminal LXXLL motifs. Defects in NR0B1 are the cause of congenital X-linked adrenal hypoplasia. Two alternatively spliced human isoforms have been described.
Protein type: Nuclear receptor
Chromosomal Location of Human Ortholog: Xp21.3
Cellular Component: nucleoplasm; polysomal ribosome; membrane; cytoplasm; nucleus
Molecular Function: DNA hairpin binding; protein domain specific binding; protein binding; ligand-dependent nuclear receptor activity; protein homodimerization activity; DNA binding; AF-2 domain binding; sequence-specific DNA binding; RNA binding; steroid hormone receptor activity; transcription corepressor activity; transcription factor binding; steroid hormone receptor binding
Biological Process: transcription initiation from RNA polymerase II promoter; negative regulation of steroid hormone receptor signaling pathway; hypothalamus development; intracellular receptor-mediated signaling pathway; gonad development; adrenal gland development; negative regulation of transcription factor activity; male gonad development; Sertoli cell differentiation; negative regulation of transcription from RNA polymerase II promoter; negative regulation of cell differentiation; protein localization; Leydig cell differentiation; pituitary gland development; male sex determination; spermatogenesis; gene expression; steroid hormone mediated signaling; negative regulation of transcription, DNA-dependent; steroid biosynthetic process
Disease: 46,xy Sex Reversal 2; Adrenal Hypoplasia, Congenital
Research Articles on NR0B1
1. We report a novel non-sense p.Gln208X mutation in the amino terminal domain of the DAX-1 gene observed in a large family with three boys presenting with adrenal manifestations at different ages.
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