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Rabbit Anti-MBNL2/BF647 Conjugated antibody (bs-18707R-BF647)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-18707R-BF647
英文名稱 Rabbit Anti-MBNL2/BF647 Conjugated antibody
中文名稱 BF647標記的盲肌樣RNA結合蛋白MBNL2抗體
別    名 DKFZp781H1296; MBLL; MBLL39; Mbnl2; MBNL2_HUMAN; MGC120625; MGC120626; MGC120628; Muscleblind like 2; muscleblind like protein 1; muscleblind like protein 2; muscleblind like protein like 39; muscleblind like splicing regulator 2; Muscleblind-like protein 1; Muscleblind-like protein 2; Muscleblind-like protein-like 39; Muscleblind-like protein-like; OTTHUMP00000178763; PRO2032; RP11-128N14.1.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 結合蛋白  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 41kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MBNL2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
This gene is a member of the muscleblind protein family which was initially described in Drosophila melanogaster. This gene encodes a C3H-type zinc finger protein that modulates alternative splicing of pre-mRNAs. Muscleblind proteins bind specifically to expanded dsCUG RNA but not to normal size CUG repeats and may thereby play a role in the pathophysiology of myotonic dystrophy. Several alternatively spliced transcript variants have been described but the full-length natures of only some have been determined. [provided by RefSeq, Mar 2012]

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. RNA-binding protein that binds to 5'ACACCC-3' core sequence, termed zipcode, within the 3'UTR of ITGA3. Binds to CUG triplet repeat expansion in myotonic dystrophy muscle cells by sequestering the target RNAs. Seems to regulates expression and localization of ITGA3 by transporting it from the nucleus to cytoplasm at adhesion plaques. May play a role in myotonic dystrophy pathophysiology (DM).

Subcellular Location:
Nucleus. Cytoplasm. Greater concentration in the nucleus. Expressed in or near large cytoplasmic adhesion plaques. Location in the cytoplasm is microtubule-dependent. In both DM1 and DM2 patients, colocalizes with nuclear foci of retained expanded-repeat transcripts.

Tissue Specificity:
Expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.

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

Database links:

Entrez Gene: 10150 Human

Entrez Gene: 105559 Mouse

Entrez Gene: 680445 Rat

Omim: 607327 Human

SwissProt: Q5VZF2 Human

SwissProt: Q8C181 Mouse

SwissProt: F2Z3T4 Rat

Unigene: 657347 Human

Unigene: 238266 Mouse

Unigene: 1940 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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