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SAB1402110

Sigma-Aldrich

Monoclonal Anti-NR0B1, (C-terminal) antibody produced in mouse

clone 1F10, purified immunoglobulin, buffered aqueous solution

Sinónimos:

AHC, AHCH, AHX, DAX-1, DAX1, DSS, GTD, HHG, NROB1

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About This Item

Código UNSPSC:
12352203
NACRES:
NA.41

origen biológico

mouse

conjugado

unconjugated

forma del anticuerpo

purified immunoglobulin

tipo de anticuerpo

primary antibodies

clon

1F10, monoclonal

formulario

buffered aqueous solution

mol peso

antigen ~38.21 kDa

reactividad de especies

human

técnicas

capture ELISA: suitable
direct immunofluorescence: suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

isotipo

IgG3κ

Nº de acceso NCBI

Nº de acceso UniProt

Condiciones de envío

dry ice

temp. de almacenamiento

−20°C

modificación del objetivo postraduccional

unmodified

Información sobre el gen

human ... NR0B1(190)

Descripción general

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)

Inmunógeno

NR0B1 (NP_000466, 361 a.a. ~ 470 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
IKCFLSKCWSLNISTKEYAYLKGTVLFNPDVPGLQCVKYIQGLQWGTQQILSEHTRMTHQGPHDRFIELNSTLFLLRFINANVIAELFFRPIIGTVSMDDMMLEMLCTKI

Acciones bioquímicas o fisiológicas

The encoded orphan nuclear receptor is involved in the growth and activity of the adrenal and reproductive organs. It is significantly associated with the growth of gonads, development of sexual characteristics and steroidogenesis. The expression of NR0B1 (nuclear receptor subfamily 0 group B member 1) gene is regulated by Nanog (transcription factor). NR0B1 is known to control pluripotency and differentiation of embryonic stem cells in mice. Mutation in NR0B1 is importantly associated with X-linked adrenal hypoplasia congenita. Mutations in NR0B1 is also known to downregulate the androgen receptor activity, also leading to defect in spermatogenesis.

Forma física

Solution in phosphate buffered saline, pH 7.4

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Código de clase de almacenamiento

10 - Combustible liquids

Clase de riesgo para el agua (WGK)

WGK 1

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°C)

Not applicable


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A Novel Mutation of DAX-1 Associated with Secretory Azoospermia.
Mou L
PLoS ONE, 10(7), 1-11 (2015)
X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism: Identification and in vitro study of a novel small indel in the NR0B1 gene.
Yu T
Molecular Medicine Reports, 13(5), 4039-4045 (2016)
Isolated 46,XY gonadal dysgenesis in two sisters caused by a Xp21.2 interstitial duplication containing the DAX1 gene.
Barbaro M
The Journal of Clinical Endocrinology and Metabolism, 92(8), 3305-3313 (2007)
A novel DAX-1 mutation in two male siblings presenting with precocious puberty and late-onset hypogonadotropic hypogonadism.
Liu Y
Journal of Pediatric Endocrinology & Metabolism : JPEM, 30(3), 349-353 (2017)
Yanxia Liu et al.
Journal of pediatric endocrinology & metabolism : JPEM, 30(3), 349-353 (2017-03-12)
Background The DAX-1 gene is associated with X-linked adrenal hypoplasia congenita (AHC). Our objective was to compare the DAX-1 gene sequence of two male siblings exhibiting different clinical manifestations of AHC. Methods Clinical features were analyzed and laboratory data were

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