Anti-Muscleblind-like 1 抗体 [EPR4565] (ab108519)

製品の概要

• 製品名Anti-Muscleblind-like 1 antibody [EPR4565]
  Muscleblind-like 1 一次抗体 製品一覧
• 製品の詳細
  Rabbit monoclonal [EPR4565] to Muscleblind-like 1
• アプリケーション適用あり: WBmore details
  適用なし: Flow Cyt,ICC,IHC-P or IP
• 種交差性
  交差種: Mouse, Human
  
• 免疫原

  Synthetic peptide corresponding to residues in Human Muscleblind-like 1.

• ポジティブ･コントロール
  • Jurkat, HeLa, and A673 cell lysates
• 特記事項

  This product is a recombinant rabbit monoclonal antibody.

  Produced using Abcam’s RabMAb^® technology. RabMAb^® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.

  Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

製品の特性

• 製品の状態Liquid
• 保存方法Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
• バッファーPBS 49%,Sodium azide 0.01%,Glycerol 50%,BSA 0.05%
• 精製度Tissue culture supernatant
• ポリ/モノモノクローナル
• クローン名EPR4565
• アイソタイプIgG
• 研究分野
  • Epigenetics and Nuclear Signaling
  • DNA / RNA
  • RNA Processing
  • Splicing

関連製品

• Positive Controls
  • HeLa nuclear extract lysate (ab14655)
  • Jurkat cytoplasmic extract lysate (ab14842)

アプリケーション

ターゲット情報

• 機能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.
• 組織特異性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.
• 関連疾患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.
• 配列類似性Belongs to the muscleblind family.
  Contains 4 C3H1-type zinc fingers.
• 細胞内局在Nucleus. Cytoplasm. Cytoplasmic granule. 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.

• Target information above from: UniProt accession Q9NR56 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt
• 参照データベース
  • Entrez Gene: 4154 Human
  • Entrez Gene: 56758 Mouse
  • Omim: 606516 Human
  • SwissProt: Q9NR56 Human
  • SwissProt: Q9JKP5 Mouse
  • Unigene: 201858 Human
  • Unigene: 725347 Human
  • Unigene: 255723 Mouse
• 別名
  • EXP antibody
  • EXP35 antibody
  • EXP40 antibody

  • EXP42 antibody
  • KIAA0428 antibody
  • MBNL antibody
  • MBNL protein antibody
  • MBNL1 antibody
  • MBNL1_HUMAN antibody
  • Muscleblind 41kD isoform antibody
  • Muscleblind like antibody
  • Muscleblind like protein 1 antibody
  • Muscleblind like splicing regulator 1 antibody
  • Muscleblind-like protein 1 antibody
  • Triplet expansion RNA binding protein antibody
  • Triplet-expansion RNA-binding protein antibody

  see all