merck millipore,默克密理愽
  • merck millipore,默克密理博,MABE70,Anti-MBNL1 Antibody, clone 4A8

  • merck millipore,默克密理博,MABE70,Anti-MBNL1 Antibody, clone 4A8

    产品名称：Anti-MBNL1 Antibody, clone 4A8
    产品型号：MABE70
    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.

    merck millipore,默克密理博,MABE70,Anti-MBNL1 Antibody, clone 4A8

  • 产品介绍
  • merck millipore,默克密理博,MABE70,Anti-MBNL1 Antibody, clone 4A8

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    HICC, WBMPurifiedMonoclonal Antibody
    描述
    产品目录编号 MABE70
    描述 Anti-MBNL1 Antibody, clone 4A8
    Alternate Names
    • Triplet-expansion RNA-binding protein
    • muscleblind-like (Drosophila)
    • muscleblind (Drosophila)-like
    • muscleblind-like protein 1
    背景信息 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.
    产品信息
    格式 Purified
    控制
    • HEK293 cell lysate
    演示 Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
    应用
    应用 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.
    主要应用
    • Immunocytochemistry
    • Western Blotting
    应用说明 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.
    生物信息
    免疫原品种 Full length recombinant protein corresponding to human MBNL1.
    克隆 4A8
    宿主 Mouse
    同种型 IgG1κ
    品种反应性 Human
    Species Reactivity Note Demonstrated to react with human.
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NP_997176
    Entrez基因汇总 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
    基因符号
    • MBNL1
    • EXP
    • MBNL
    • EXP35
    • EXP40
    • EXP42
    • KIAA0428
    • DKFZp686P06174
    纯化方法 Protein G
    UniProt编号
    • Q9NR56
    UniProt汇总 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.
    产品使用声明
    质量保证 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.
    使用声明
    • 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.
    储存和货运信息
    存储条件 Stable for 1 year at 2-8°C from date of receipt.
    包装信息
    数量 100 µL

    merck millipore,默克密理博,MABE70,Anti-MBNL1 Antibody, clone 4A8

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