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ABE241 Anti-MBNL1 Antibody

Use Anti-MBNL1 Antibody (Rabbit Polyclonal Antibody) validated in WB to detect MBNL1 also known as muscleblind-like (Drosophila), Triplet-expansion RNA-binding protein.

Anti-MBNL1 Antibody: MSDS (material safety data sheet) o SDS, certificato d’analisi (CoA) e certificato di qualità (CoQ), dossier, brochure e altri documenti disponibili.
Research Category: Epigenetics & Nuclear Function Research Sub-Category: RNA Metabolism & Binding Proteins Gene Symbol: EXP, MBNL, MBNL1, EXP42, EXP35, EXP40 UniProt Number: Q9NR56
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ABE241
100 µg  
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      Tabella delle specifiche principali

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, RWBRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue NumberABE241
      DescriptionAnti-MBNL1 Antibody
      Alternate Names
      • muscleblind-like (Drosophila)
      • Triplet-expansion RNA-binding protein
      • muscleblind (Drosophila)-like
      • muscleblind-like protein 1
      • KIAA0428
      Background InformationMuscleblind 1 (MBNL1) is a member of the muscleblind family. MBNL1in 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.
      References
      Product Information
      FormatAffinity Purified
      Control
      • A431 cell lysate
      PresentationPurified rabbit polyclonal in tris-citrate containing 0.1% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationUse Anti-MBNL1 Antibody (Rabbit Polyclonal Antibody) validated in WB to detect MBNL1 also known as muscleblind-like (Drosophila), Triplet-expansion RNA-binding protein.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenHistidine-tagged recombinant protein corresponding to human MBNL1.
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRabbit
      Species Reactivity
      • Human
      • Mouse
      • Rat
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • EXP
      • MBNL
      • MBNL1
      • EXP42
      • EXP35
      • EXP40
      Purification MethodAffinity Purfied
      UniProt Number
      UniProt SummaryFUNCTION: 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.

      SUBUNIT STRUCTURE: Interacts with DDX1 and YBX1. Interacts with HNRNPH1; the interaction in RNA-independent.

      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.

      TISSUE SPECIFICTY: 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.

      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.

      SEQUENCE CAUTION: The sequence BAA24858.2 differs from that shown. Reason: Erroneous initiation.
      Molecular Weight~ 33-42 kDa observed. There are three known splice variants ranging from ~33 kDa to ~42 kDa. An uncharacterized band at ~ 80 kDa has been observed in some lysates.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in A431 cell lysate.

      Western Blot Analysis: 1 µg/mL of this antibody detected MBNL1 on 10 µg of A431 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 ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Numero di catalogo GTIN
      ABE241 04053252642531

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      Categorie

      Life Science Research > Antibodies and Assays > Primary Antibodies