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Key Documents

MABE70

Sigma-Aldrich

Anti-MBNL1 Antibody, clone 4A8

clone 4A8, from mouse

Synonym(e):

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

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About This Item

UNSPSC-Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

Biologische Quelle

mouse

Qualitätsniveau

100

Antikörperform

purified immunoglobulin

Antikörper-Produkttyp

primary antibodies

Klon

4A8, monoclonal

Speziesreaktivität

human

Methode(n)

immunocytochemistry: suitable
western blot: suitable

Isotyp

IgG1κ

NCBI-Hinterlegungsnummer

NP_997176

UniProt-Hinterlegungsnummer

Q9NR56

Versandbedingung

wet ice

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... MBNL1(4154)

Allgemeine Beschreibung

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.

Immunogen

Full length recombinant protein corresponding to human MBNL1.

Anwendung

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.
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.

Qualität

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.

Zielbeschreibung

~39 kDa observed

Physikalische Form

Format: Purified

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Lagerklassenschlüssel

12 - Non Combustible Liquids

WGK

WGK 1

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable

Analysenzertifikate (COA)

Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.

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Die Dokumentenbibliothek aufrufen

Raphael I Benhamou et al.
Cell chemical biology, 27(2), 223-231 (2020-01-26)
Myotonic dystrophy type 2 (DM2) is a genetically defined disease caused by a toxic expanded repeat of r(CCUG) [r(CCUG)exp], harbored in intron 1 of CCHC-type zinc-finger nucleic acid binding protein (CNBP) pre-mRNA. This r(CCUG)exp causes toxicity via a gain-of-function mechanism
Eric T Wang et al.
Cell, 150(4), 710-724 (2012-08-21)
The muscleblind-like (Mbnl) family of RNA-binding proteins plays important roles in muscle and eye development and in myotonic dystrophy (DM), in which expanded CUG or CCUG repeats functionally deplete Mbnl proteins. We identified transcriptome-wide functional and biophysical targets of Mbnl
Raphael I Benhamou et al.
Journal of medicinal chemistry, 63(14), 7827-7839 (2020-07-14)
RNA repeat expansions are responsible for more than 30 incurable diseases. Among them is myotonic dystrophy type 1 (DM1), the most common form of adult on-set muscular dystrophy. DM1 is caused by an r(CUG) repeat expansion [r(CUG)exp] located in the
Ian Holt et al.
Genes to cells : devoted to molecular & cellular mechanisms, 12(9), 1035-1048 (2007-09-11)
Nuclear speckles are storage sites for small nuclear RNPs (snRNPs) and other splicing factors. Current ideas about the role of speckles suggest that some pre-mRNAs are processed at the speckle periphery before being exported as mRNA. In myotonic dystrophy type
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)

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