생물학적 소스
rabbit
Quality Level
결합
unconjugated
항체 형태
affinity isolated antibody
항체 생산 유형
primary antibodies
클론
polyclonal
제품 라인
Prestige Antibodies® Powered by Atlas Antibodies
형태
buffered aqueous glycerol solution
종 반응성
human
향상된 검증
recombinant expression
Learn more about Antibody Enhanced Validation
기술
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
western blot: 0.04-0.4 μg/mL
면역원 서열
PVALQTRLAKRGILKHLEPEPEEEIIAEDYDDDPVDYEATRLEGLPPSWYKVFDPSCGLPYYWNADTDLVSWLSPHDPNSVVTKSAKKLRSSNADAEEKLDRSHDKSDRGHDKSDRSHEKLDRGHDKSDRGHDKSDRDRERGYDKVD
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... PQBP1(10084)
일반 설명
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.
면역원
Polyglutamine-binding protein 1 recombinant protein epitope signature tag (PrEST)
애플리케이션
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.
생화학적/생리학적 작용
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.
특징 및 장점
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.
결합
Corresponding Antigen APREST74292
물리적 형태
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
법적 정보
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
면책조항
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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Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
개인 보호 장비
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
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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