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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

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Synonym(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
MDL number:
MFCD09039233
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

Quality Level

200

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

HL 1822 (3A4-1E9), monoclonal

form

buffered aqueous solution

mol wt

antigen ~42 kDa

species reactivity

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

UniProt accession no.

Q9NR56

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

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

Related Categories

General description

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.

Immunogen

recombinant muscleblind-like1 (MBNL1) fusion protein.

Application

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

Biochem/physiol Actions

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.

Physical form

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

Disclaimer

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.

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

Regulatory Information

常规特殊物品

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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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)
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.
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