HPA001880
Anti-PQBP1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinónimos:
Anti-MRX2, Anti-MRX55, Anti-MRXS8, Anti-RENS1, Anti-SHS
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About This Item
origen biológico
rabbit
Nivel de calidad
conjugado
unconjugated
forma del anticuerpo
affinity isolated antibody
tipo de anticuerpo
primary antibodies
clon
polyclonal
Línea del producto
Prestige Antibodies® Powered by Atlas Antibodies
Formulario
buffered aqueous glycerol solution
reactividad de especies
human
validación mejorada
recombinant expression
Learn more about Antibody Enhanced Validation
técnicas
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
western blot: 0.04-0.4 μg/mL
secuencia del inmunógeno
PVALQTRLAKRGILKHLEPEPEEEIIAEDYDDDPVDYEATRLEGLPPSWYKVFDPSCGLPYYWNADTDLVSWLSPHDPNSVVTKSAKKLRSSNADAEEKLDRSHDKSDRGHDKSDRSHEKLDRGHDKSDRGHDKSDRDRERGYDKVD
Nº de acceso UniProt
Condiciones de envío
wet ice
temp. de almacenamiento
−20°C
modificación del objetivo postraduccional
unmodified
Información sobre el gen
human ... PQBP1(10084)
Categorías relacionadas
Descripción general
Polyglutamine tract binding protein 1 (PQBP1) with a WW domain plays an important role in regulation of transcription activity. WW domain binds to proline-rich motifs in other proteins. In addition to the WW domain, it also possesses a polyglutamine-binding region (in polar-amino-acid–rich domain) and a DR/ER stretch involved in transcriptional control. It is ubiquitously expressed in neurons throughout the brain specifically in hippocampus, cerebellar cortex and olfactory bulb. It localizes in the nucleus.
Inmunógeno
Polyglutamine-binding protein 1 recombinant protein epitope signature tag (PrEST)
Aplicación
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Acciones bioquímicas o fisiológicas
During transcriptional control, PQBP1 (polyglutamine binding protein 1) binds to the polyQ region of the transcription factor BRN2 (brain-specific homeobox/POU domain protein 2) to silences transcription. Mutation in PQBP1 causes X-linked mental retardation, microcephaly, short stature, spastic paraplegia and midline defects. It interacts with RNA polymerase II and other transcription factors. In addition, it has been reported in RNA transport or cytoplasmic RNA processing.
Características y beneficios
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Ligadura / enlace
Corresponding Antigen APREST74292
Forma física
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Información legal
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Cláusula de descargo de responsabilidad
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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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
Equipo de protección personal
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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Certificados de análisis (COA)
Lot/Batch Number
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Encuentre la documentación para los productos que ha comprado recientemente en la Biblioteca de documentos.
H Okazawa et al.
Brain research bulletin, 56(3-4), 273-280 (2001-11-24)
Polyglutamine(Q) tract binding protein-1 (PQBP-1) was isolated on the basis of its interaction with polyglutamine tracts and localizes predominantly to the nucleus where it suppresses transcriptional activation by a neuron-specific transcription factor, Brn-2. Its C-terminal domain is highly conserved and
Vera M Kalscheuer et al.
Nature genetics, 35(4), 313-315 (2003-11-25)
We found mutations in the gene PQBP1 in 5 of 29 families with nonsyndromic (MRX) and syndromic (MRXS) forms of X-linked mental retardation (XLMR). Clinical features in affected males include mental retardation, microcephaly, short stature, spastic paraplegia and midline defects.
Novel truncating mutations in the polyglutamine tract binding protein 1 gene (PQBP1) cause Renpenning syndrome and X-linked mental retardation in another family with microcephaly.
Claus Lenski et al.
American journal of human genetics, 74(4), 777-780 (2004-03-17)
Qingqing Wang et al.
Genes & development, 27(6), 615-626 (2013-03-21)
Polyglutamine-binding protein 1 (PQBP1) is a highly conserved protein associated with neurodegenerative disorders. Here, we identify PQBP1 as an alternative messenger RNA (mRNA) splicing (AS) effector capable of influencing splicing of multiple mRNA targets. PQBP1 is associated with many splicing
M Waragai et al.
Human molecular genetics, 8(6), 977-987 (1999-05-20)
A novel gene, designated PQBP-1, which encodes a 265 residue protein that binds to the polyglutamine tract of the brain-specific transcription factor Brn-2, was identified. PQBP-1, which also interacts with the polyglutamine tract of triplet repeat disease gene products, binds
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