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Merck
모든 사진(5)

주요 문서

WH0007337M1

Sigma-Aldrich

Monoclonal Anti-UBE3A antibody produced in mouse

clone 2F6, purified immunoglobulin, buffered aqueous solution

동의어(들):

Anti-ANCR, Anti-AS, Anti-E6AP, Anti-EPVE6AP, Anti-HPVE6A, Anti-ubiquitin protein ligase E3A (human papilloma virus E6-associated protein, Angelman syndrome)

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About This Item

MDL number:
UNSPSC 코드:
12352203
NACRES:
NA.41
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생물학적 소스

mouse

결합

unconjugated

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

2F6, monoclonal

양식

buffered aqueous solution

종 반응성

human

기술

immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
indirect immunofluorescence: suitable
western blot: 1-5 μg/mL

동형

IgG2aκ

GenBank 수납 번호

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... UBE3A(7337)

일반 설명

This gene encodes an E3 ubiquitin-protein ligase, part of the ubiquitin protein degradation system. This imprinted gene is maternally expressed in brain and biallelically expressed in other tissues. Maternally inherited deletion of this gene causes Angelman Syndrome, characterized by severe motor and intellectual retardation, ataxia, hypotonia, epilepsy, absence of speech, and characteristic facies. The protein also interacts with the E6 protein of human papillomavirus types 16 and 18, resulting in ubiquitination and proteolysis of tumor protein p53. Alternative splicing of this gene results in three transcript variants encoding three isoforms with different N-termini. Additional transcript variants have been described, but their full length nature has not been determined. (provided by RefSeq)

면역원

UBE3A (AAH09271, 51 a.a. ~ 150 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
ETFQQLITYKVISNEFNSRNLVNDDDAIVAASKCLKMVYYANVVGGEVDTNHNEEDDEEPIPESSELTLQELLGEERRNKKGPRVDPLETELGVKTLDCR

생화학적/생리학적 작용

The ubiquitin protein ligase E3A (UBE3A) functions as E3 ligase in the ubiquitin-proteasome pathway and act as transcriptional coactivator of steroid hormone receptors such as progesterone, estrogen and other hormone receptors. During papilloma virus infection, ubiquitin protein ligase E3A (UBE3A), along with E6 viral protein, plays a vital role in degradation of the cell cycle regulatory protein p53. Anomalistic degradation of p53 can cause cervical cancer. Inactivation or deletion of UBE3A gene leads to the neurodevelopmental disorder Angelman syndrome (AS), while overexpression of maternal UBE3A gene is associated with autism pathogenesis. Therefore UBE3A level has to be properly controlled for normal brain development.

물리적 형태

Solution in phosphate buffered saline, pH 7.4

법적 정보

GenBank is a registered trademark of United States Department of Health and Human Services

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

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Lot/Batch Number

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문서 라이브러리 방문

Catalytically Important Residues of E6AP Ubiquitin Ligase Identified Using Acid-Cleavable Photo-Cross-Linkers.
Krist DT and Statsyuk AV
Biochemistry, 54(29), 4411-4414 (2015)
An Autism-Linked Mutation Disables Phosphorylation Control of UBE3A.
Yi JJ
Cell, 162(4), 795-807 (2015)
Epigenetic regulation of UBE3A and roles in human neurodevelopmental disorders.
LaSalle JM
Epigenomics, 7(7), 1213-1228 (2015)
The Angelman syndrome ubiquitin ligase localizes to the synapse and nucleus, and maternal deficiency results in abnormal dendritic spine morphology.
Dindot SV
Human Molecular Genetics, 17(1), 111-118 (2008)

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