Anti-MBNL1, clone 4A8 (mouse monoclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
H	WB, ICC	M	Purified	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

WB, ICC

Purified

Monoclonal Antibody

Description
Catalogue Number	MABE70
Description	Anti-MBNL1 Antibody, clone 4A8
Alternate Names	muscleblind-like (Drosophila) muscleblind (Drosophila)-like muscleblind-like protein 1 Triplet-expansion RNA-binding protein
Background Information	Muscleblind 1 (MBNL1) is a member of the muscleblind family. MBNL1 is involved in pre-mRNA alternative splicing and acts as either splicing activator or repressor on specific pre-mRNA targets. This protein contains four C3H1-type zinc fingers and binds to expanded CUG repeat RNA. High expression of MBNL1 has been observed in cardiac and skeletal muscle as well as in differentiating myoblasts. MBNL1 is known to be involved in the pathogenesis of dystrophia myotonica type 1 (DM1). For those afflicted with this muscular disorder, MBNL1 is sequestered by DMPK RNAs that contain CUG triplet repeat expansions.

Description

Catalogue Number

MABE70

Description

Anti-MBNL1 Antibody, clone 4A8

Alternate Names

muscleblind-like (Drosophila)
muscleblind (Drosophila)-like
muscleblind-like protein 1
Triplet-expansion RNA-binding protein

Background Information

Muscleblind 1 (MBNL1) is a member of the muscleblind family. MBNL1 is involved in pre-mRNA alternative splicing and acts as either splicing activator or repressor on specific pre-mRNA targets. This protein contains four C3H1-type zinc fingers and binds to expanded CUG repeat RNA. High expression of MBNL1 has been observed in cardiac and skeletal muscle as well as in differentiating myoblasts. MBNL1 is known to be involved in the pathogenesis of dystrophia myotonica type 1 (DM1). For those afflicted with this muscular disorder, MBNL1 is sequestered by DMPK RNAs that contain CUG triplet repeat expansions.

Product Information
Format	Purified
Control	HEK293 cell lysate
Presentation	Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Product Information

Format

Purified

Control

HEK293 cell lysate

Presentation

Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Applications
Application	Anti-MBNL1 Antibody, clone 4A8 is a Mouse Monoclonal Antibody for detection of MBNL1 also known as muscleblind-like protein 1, Triplet-expansion RNA-binding protein & has been validated in WB, ICC.
Key Applications	Western Blotting Immunocytochemistry
Application Notes	Immunocytochemistry Analysis: 1:4 dilution from a representative lot detected MBNL1 in myotonic dystrophy cells. Data courtesy of Dr. Ian Holt, Wolfson Centre for Inherited Neuromuscular Disease.

Applications

Application

Anti-MBNL1 Antibody, clone 4A8 is a Mouse Monoclonal Antibody for detection of MBNL1 also known as muscleblind-like protein 1, Triplet-expansion RNA-binding protein & has been validated in WB, ICC.

Key Applications

Western Blotting
Immunocytochemistry

Application Notes

Immunocytochemistry Analysis:
1:4 dilution from a representative lot detected MBNL1 in myotonic dystrophy cells. Data courtesy of Dr. Ian Holt, Wolfson Centre for Inherited Neuromuscular Disease.

Biological Information
Immunogen	Full length recombinant protein corresponding to human MBNL1.
Clone	4A8
Host	Mouse
Isotype	IgG1κ
Species Reactivity	Human
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NP_997176
Entrez Gene Summary	Official Symbol: MBNL1provided by HGNC Official Full Name: muscleblind-like (Drosophila)provided by HGNC Primary source: HGNC:6923 See related: Ensembl:ENSG00000152601; HPRD:07355; MIM:606516 Gene type: protein coding RefSeq status: VALIDATED Organism: Homo sapiens Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as: EXP; MBNL; EXP35; EXP40; EXP42; KIAA0428; DKFZp686P06174; MBNL1
Gene Symbol	EXP MBNL EXP35 EXP40 EXP42 KIAA0428 DKFZp686P06174 MBNL1
Purification Method	Protein G
UniProt Number	Q9NR56
UniProt Summary	Function: Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues. Ref.3 Ref.5 Ref.6 Ref.7 Ref.8 Subunit structure: Interacts with DDX1 and YBX1. Interacts with HNRNPH1; the interaction in RNA-independent. Ref.6 Ref.7 Subcellular location: Nucleus. Cytoplasm. Cytoplasmic granule. Note: Localized with DDX1, TIAL1 and YBX1 in stress granules upon stress. Localized in the cytoplasm of multinucleated myotubes. Colocalizes with nuclear foci of retained expanded-repeat transcripts in myotubes from patients affected by myotonic dystrophy. Ref.3 Ref.7 Ref.4 Tissue specificity: Highly expressed in cardiac, skeletal muscle and during myoblast differentiation. Weakly expressed in other tissues (at protein level). Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Ref.3 Ref.4 Involvement in disease: Plays a role in the pathogenesis of dystrophia myotonica type 1 (DM1) [MIM:160900]. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=In muscle cells from DM1 patients, MBNL1 is sequestered by DMPK RNAs containing CUG triplet repeat expansions. MBNL1 binding is proportional to repeat length consistent with the direct correlation between the length of repeat expansion and disease severity. Sequence similarities: Belongs to the muscleblind family. Contains 4 C3H1-type zinc fingers.
Molecular Weight	~39 kDa observed

Biological Information

Immunogen

Full length recombinant protein corresponding to human MBNL1.

Clone

4A8

Host

Mouse

Isotype

IgG1κ

Species Reactivity

Human

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NP_997176

Entrez Gene Summary

Official Symbol: MBNL1provided by HGNC
Official Full Name: muscleblind-like (Drosophila)provided by HGNC
Primary source: HGNC:6923
See related: Ensembl:ENSG00000152601; HPRD:07355; MIM:606516
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Homo sapiens
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as: EXP; MBNL; EXP35; EXP40; EXP42; KIAA0428; DKFZp686P06174; MBNL1

Gene Symbol

EXP
MBNL
EXP35
EXP40
EXP42
KIAA0428
DKFZp686P06174
MBNL1

Purification Method

Protein G

UniProt Number

Q9NR56

UniProt Summary

Function: Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues. Ref.3 Ref.5 Ref.6 Ref.7 Ref.8

Subunit structure: Interacts with DDX1 and YBX1. Interacts with HNRNPH1; the interaction in RNA-independent. Ref.6 Ref.7

Subcellular location: Nucleus. Cytoplasm. Cytoplasmic granule. Note: Localized with DDX1, TIAL1 and YBX1 in stress granules upon stress. Localized in the cytoplasm of multinucleated myotubes. Colocalizes with nuclear foci of retained expanded-repeat transcripts in myotubes from patients affected by myotonic dystrophy. Ref.3 Ref.7 Ref.4

Tissue specificity: Highly expressed in cardiac, skeletal muscle and during myoblast differentiation. Weakly expressed in other tissues (at protein level). Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Ref.3 Ref.4

Involvement in disease: Plays a role in the pathogenesis of dystrophia myotonica type 1 (DM1) [MIM:160900]. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=In muscle cells from DM1 patients, MBNL1 is sequestered by DMPK RNAs containing CUG triplet repeat expansions. MBNL1 binding is proportional to repeat length consistent with the direct correlation between the length of repeat expansion and disease severity.

Sequence similarities: Belongs to the muscleblind family.

Contains 4 C3H1-type zinc fingers.

Molecular Weight

~39 kDa observed

Product Usage Statements
Quality Assurance	Evaluated by Western Blot in HEK293 cell lysate. Western Blot Analysis: 1:10,000 dilution of this antibody detected MBNL1 on 10 µg of HEK293 cell lysate.
Usage Statement	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.

Product Usage Statements

Quality Assurance

Evaluated by Western Blot in HEK293 cell lysate.

Western Blot Analysis: 1:10,000 dilution of this antibody detected MBNL1 on 10 µg of HEK293 cell lysate.

Usage Statement

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.

Storage and Shipping Information
Storage Conditions	Stable for 1 year at 2-8°C from date of receipt.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at 2-8°C from date of receipt.

Packaging Information
Material Size	100 µL

Packaging Information

Material Size

100 µL

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Anti-MBNL1, clone 4A8 (mouse monoclonal)

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