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Merck
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文件

M3320

Sigma-Aldrich

Monoclonal Anti-MBNL1 antibody produced in mouse

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

别名:

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號碼:
MFCD09039233
分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

mouse

品質等級

200

共軛

unconjugated

抗體表格

purified immunoglobulin

抗體產品種類

primary antibodies

無性繁殖

HL 1822 (3A4-1E9), monoclonal

形狀

buffered aqueous solution

分子量

antigen ~42 kDa

物種活性

monkey, mouse, human

濃度

~1 mg/mL

技術

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

同型

IgG1

UniProt登錄號

Q9NR56

運輸包裝

dry ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

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

相关类别

一般說明

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.

免疫原

recombinant muscleblind-like1 (MBNL1) fusion protein.

應用

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

生化／生理作用

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.

外觀

0.01M 磷酸缓冲盐溶液，pH 7.4，含 15mM 叠氮化钠。

免責聲明

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.

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儲存類別代碼

10 - Combustible liquids

水污染物質分類（WGK）

WGK 3

閃點（°F）

Not applicable

閃點（°C）

Not applicable

個人防護裝備

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

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Nan Zhang et al.
Frontiers in genetics, 11, 594576-594576 (2020-12-29)
Cas13a, an effector of type VI CRISPR-Cas systems, is an RNA guided RNase with multiplexing and therapeutic potential. This study employs the Leptotrichia shahii (Lsh) Cas13a and a repeat-based CRISPR RNA (crRNA) to track and eliminate toxic RNA aggregates in
The Muscleblind family of proteins: an emerging class of regulators of developmentally programmed alternative splicing
Pascual M, et al.
Differentiation, 74, 65-80 (2006)
Fan Zhang et al.
Human molecular genetics, 26(16), 3056-3068 (2017-05-24)
Myotonic dystrophy Type 1 (DM1) is a rare genetic disease caused by the expansion of CTG trinucleotide repeats ((CTG)exp) in the 3' untranslated region of the DMPK gene. The repeat transcripts sequester the RNA binding protein Muscleblind-like protein 1 (MBNL1)
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.
A Muscleblind Knockout Model for Myotonic Dystrophy
Kanadia RN, et al.
Science, 302, 1978-1980 (2003)
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