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MBNL3 Rabbit pAb (bs-5721R)  
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產品編號 bs-5721R
英文名稱 MBNL3 Rabbit pAb
中文名稱 毒蕈堿樣蛋白3抗體
別    名 MBLX; CHCR; Cys3His CCG1 required protein; MBLX39; MBNL 3; MBXL; Muscleblind like protein 3; Muscleblind like X linked protein; Protein HCHCR; MBNL3_HUMAN.  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  細胞周期蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse (predicted: Human,Rat,Pig,Sheep,Cow,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 39 kDa
檢測分子量
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MBNL3: 251-354/354 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 MBNL3 has been shown to inhibit muscle differentiation. MBNL3 belongs to a highly conserved family of tissue-specific alternative splicing regulators. This family is known to regulate terminal muscle differentiation through alternative splicing control and several groups have suggested that the family participates in the differentiation of photoreceptors, neurons, adipocytes and blood cell types. MBNL3 is expressed in myoblasts, muscle precursor cells, and during the early stages of myogenesis, but is detected at very low levels in terminally differentiated myotubes. MBNL proteins have been shown to sequester foci of expanded-repeat transcripts and are thought to therefore play a role in the molecular pathology of a group of neuromuscular diseases including the Myotonic Dystrophies.

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. May play a role in myotonic dystrophy pathophysiology (DM). Could inhibit terminal muscle differentiation, acting at approximately the time of myogenin induction.

Subcellular Location:
Nucleus. Cytoplasm. Note=Greater concentration in the nucleus. In both DM1 and DM2 patients, colocalizes with nuclear foci of retained expanded-repeat transcripts.

Tissue Specificity:
Highly expressed in the placenta.

Similarity:
Belongs to the muscleblind family.
Contains 4 C3H1-type zinc fingers.

SWISS:
Q9NUK0

Gene ID:
55796

Database links:

Entrez Gene: 55796 Human

Entrez Gene: 171170 Mouse

Entrez Gene: 302492 Rat

Omim: 300413 Human

SwissProt: Q9NUK0 Human

SwissProt: Q8R003 Mouse

Unigene: 105134 Human

Unigene: 596347 Human

Unigene: 295324 Mouse

Unigene: 105030 Rat



產品圖片
Paraformaldehyde-fixed, paraffin embedded (Mouse placenta); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MBNL3) Polyclonal Antibody, Unconjugated (bs-5721R) at 1:500 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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