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M3320

Sigma-Aldrich

Monoclonal Anti-MBNL1 antibody produced in mouse

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

Synonyme(s) :

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

Numéro MDL:
MFCD09039233
Code UNSPSC :
12352203
Nomenclature NACRES :
NA.41

Source biologique

mouse

Niveau de qualité

200

Conjugué

unconjugated

Forme d'anticorps

purified immunoglobulin

Type de produit anticorps

primary antibodies

Clone

HL 1822 (3A4-1E9), monoclonal

Forme

buffered aqueous solution

Poids mol.

antigen ~42 kDa

Espèces réactives

monkey, mouse, human

Concentration

~1 mg/mL

Technique(s)

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

Isotype

IgG1

Numéro d'accès UniProt

Q9NR56

Conditions d'expédition

dry ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

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

Description générale

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.

Immunogène

recombinant muscleblind-like1 (MBNL1) fusion protein.

Application

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

Actions biochimiques/physiologiques

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.

Forme physique

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

Clause de non-responsabilité

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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Code de la classe de stockage

10 - Combustible liquids

Classe de danger pour l'eau (WGK)

WGK 3

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Équipement de protection individuelle

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

Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Consulter la Bibliothèque de documents

The Muscleblind family of proteins: an emerging class of regulators of developmentally programmed alternative splicing
Pascual M, et al.
Differentiation, 74, 65-80 (2006)
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.
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)
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
Muscleblind-like 1 (Mbnl1) regulates pre-mRNA alternative splicing during terminal erythropoiesis
Cheng AW, et al.
Blood, 124, 598-610 (2014)
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