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Merck
모든 사진(2)

Key Documents

M3320

Sigma-Aldrich

Monoclonal Anti-MBNL1 antibody produced in mouse

~1 mg/mL, clone HL 1822 (3A4-1E9), purified immunoglobulin, buffered aqueous solution

동의어(들):

Anti-CUG Triplet Repeat Expansion Double-stranded RNA-binding protein, Anti-EXP, Anti-KIAA0428, Anti-Muscleblind-like protein, Anti-Triplet-expansion RNA-binding protein

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About This Item

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

생물학적 소스

mouse

Quality Level

결합

unconjugated

항체 형태

purified immunoglobulin

항체 생산 유형

primary antibodies

클론

HL 1822 (3A4-1E9), monoclonal

형태

buffered aqueous solution

분자량

antigen ~42 kDa

종 반응성

monkey, mouse, human

농도

~1 mg/mL

기술

immunocytochemistry: suitable
immunohistochemistry: suitable
microarray: suitable
western blot: 0.2-0.5 μg/mL using HeLa nuclear cell extract

동형

IgG1

UniProt 수납 번호

배송 상태

dry ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... MBNL1(4154)
mouse ... Mbnl1(56758)

일반 설명

Muscle blind-like 1 (MBNL1) is a homolog of human muscle blind like proteins. Proteins of the MBNL (muscleblind-like) family are mainly expressed in skeletal muscle neuronal tissues, thymus, liver and kidney.

면역원

recombinant muscleblind-like1 (MBNL1) fusion protein.

애플리케이션

Monoclonal Anti-MBNL1 antibody produced in mouse has been used in: immunoblotting, immunohistochemistry and immunocytochemistry

생화학적/생리학적 작용

Proteins of the MBNL (muscleblind-like) family enhance inclusion or exclusion of specific exons on different pre-mRNAs by inhibiting the activity of CUG-BP and ETR-3-like factors (CELF proteins) bound to distinct intronic sites. MBNL1 may be implicated in the stimulation of muscle differentiation. Mice knockouts for the MBNL1 gene develop muscle, eye, and RNA splicing abnormalities that are characteristic of the neuromuscular disease myotonic dystrophy (DM) disease.

물리적 형태

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

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 3

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

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


시험 성적서(COA)

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문서 라이브러리 방문

The Muscleblind family of proteins: an emerging class of regulators of developmentally programmed alternative splicing
Pascual M, et al.
Differentiation, 74, 65-80 (2006)
Muscleblind-like 1 (Mbnl1) regulates pre-mRNA alternative splicing during terminal erythropoiesis
Cheng AW, et al.
Blood, 124, 598-610 (2014)
A Muscleblind Knockout Model for Myotonic Dystrophy
Kanadia RN, et al.
Science, 302, 1978-1980 (2003)
Curtis A Nutter et al.
Muscle & nerve, 56(4), 744-749 (2017-02-07)
Type 1 diabetic patients can develop skeletal muscle weakness and atrophy by molecular mechanisms that are not well understood. Alternative splicing (AS) is critical for gene expression in the skeletal muscle, and its dysregulation is implicated in muscle weakness and
Debra A O'Leary et al.
Current chemical genomics, 4, 9-18 (2010-05-27)
Myotonic dystrophy type 1 (DM1) is a genetic disorder characterized by muscle wasting, myotonia, cataracts, cardiac arrhythmia, hyperinsulinism and intellectual deficits, and is caused by expansion of a CTG repeat in the 3'UTR of the Dystrophia Myotonica-Protein Kinase (DMPK) gene.

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