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merck millipore,默克密理博,ABE241,Anti-MBNL1 Antibody
产品名称：Anti-MBNL1 Antibody
产品型号：ABE241
Use Anti-MBNL1 Antibody (Rabbit Polyclonal Antibody) validated in WB to detect MBNL1 also known as muscleblind-like (Drosophila), Triplet-expansion RNA-binding protein.
merck millipore,默克密理博,ABE241,Anti-MBNL1 Antibody
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 ABE241 描述 Anti-MBNL1 Antibody Alternate Names - KIAA0428
- muscleblind-like (Drosophila)
- Triplet-expansion RNA-binding protein
- muscleblind (Drosophila)-like
- muscleblind-like protein 1
背景信息 Muscleblind 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. 产品信息 格式 Affinity Purified 控制 - A431 cell lysate
演示 Purified rabbit polyclonal in tris-citrate containing 0.1% sodium azide. 应用 应用 Use Anti-MBNL1 Antibody (Rabbit Polyclonal Antibody) validated in WB to detect MBNL1 also known as muscleblind-like (Drosophila), Triplet-expansion RNA-binding protein. 主要应用 - Western Blotting
生物信息 免疫原品种 Histidine-tagged recombinant protein corresponding to human MBNL1. 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 品种反应性 RatHumanMouse Species Reactivity Note Demonstrated to react with human, mouse, and rat. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_066368
基因符号 - EXP40
- EXP
- MBNL
- MBNL1
- EXP42
- EXP35
纯化方法 Affinity purified 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.
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.产品使用声明 质量保证 Evaluated 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.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 µg
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