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WH0007337M1

Sigma-Aldrich

Monoclonal Anti-UBE3A antibody produced in mouse

clone 2F6, purified immunoglobulin, buffered aqueous solution

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别名:
Anti-ANCR, Anti-AS, Anti-E6AP, Anti-EPVE6AP, Anti-HPVE6A, Anti-ubiquitin protein ligase E3A (human papilloma virus E6-associated protein, Angelman syndrome)
MDL编号:
NACRES:
NA.41

生物来源

mouse

质量水平

偶联物

unconjugated

抗体形式

purified immunoglobulin

antibody product type

primary antibodies

克隆

2F6, monoclonal

形式

buffered aqueous solution

species reactivity

human

technique(s)

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

同位素/亚型

IgG2aκ

GenBank® accession no.

UniProt登记号

运输

dry ice

储存温度

−20°C

target post-translational modification

unmodified

Gene Information

human ... UBE3A(7337)

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WH0007327M1WH0007314M1WH0084254M1
conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody form

purified immunoglobulin

antibody form

purified immunoglobulin

antibody form

purified immunoglobulin

clone

2F6, monoclonal

clone

5E1, monoclonal

clone

1F5, monoclonal

clone

1F6, monoclonal

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

species reactivity

human

species reactivity

human

species reactivity

human

species reactivity

human

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一般描述

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)

免疫原

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

生化/生理作用

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.

外形

Solution in phosphate buffered saline, pH 7.4

法律信息

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

免责声明

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.

储存分类代码

10 - Combustible liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable

个人防护装备

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

法规信息

常规特殊物品

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An Autism-Linked Mutation Disables Phosphorylation Control of UBE3A.
Yi JJ
Cell, 162(4), 795-807 (2015)
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)
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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