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Rabbit Anti-MBNL2/BF350 Conjugated antibody (bs-18707R-BF350)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-18707R-BF350
英文名称 Rabbit Anti-MBNL2/BF350 Conjugated antibody
中文名称 BF350标记的盲肌样RNA结合蛋白MBNL2抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 结合蛋白  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, )
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MBNL2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件 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.
产品介绍 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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