产品货号 :  mlR18707

英文名称 :  MBNL2

中文名称 :  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.  

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 :  Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  41kDa

细胞定位 :  细胞核 细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human MBNL2:101-200/373

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.

PubMed :  PubMed

产品介绍 :  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.

SWISS:

Q5VZF2

Gene ID:

10150

Important Note:

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