Recombinant Mouse Muscleblind-like protein 1 (Mbnl1)

中文名稱：

小鼠Mbnl1重組蛋白
貨號：

CSB-YP867516MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Mbnl1重組蛋白
貨號：

CSB-EP867516MO
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

小鼠Mbnl1重組蛋白
貨號：

CSB-EP867516MO-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

小鼠Mbnl1重組蛋白
貨號：

CSB-BP867516MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Mbnl1重組蛋白
貨號：

CSB-MP867516MO
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Mbnl1
Uniprot No.：

Q9JKP5
別名：

Mbnl1; Exp; MbnlMuscleblind-like protein 1; Triplet-expansion RNA-binding protein
種屬：

Mus musculus (Mouse)
蛋白長度：

full length protein
表達區域：

1-341
氨基酸序列

MAVSVTPIRD TKWLTLEVCR EFQRGTCSRP DTECKFAHPS KSCQVENGRV IACFDSLKGR CSRENCKYLH PPPHLKTQLE INGRNNLIQQ KNMAMLAQQM QLANAMMPGA PLQPVPMFSV APSLATSASA AFNPYLGPVS PSLVPAEILP TAPMLVTGNP GVPVPAAAAA AAQKLMRTDR LEVCREYQRG NCNRGENDCR FAHPADSTMI DTNDNTVTVC MDYIKGRCSR EKCKYFHPPA HLQAKIKAAQ YQVNQAAAAQ AAATAAAMGI PQAVLPPLPK RPALEKTNGA TAVFNTGIFQ YQQALANMQL QQHTAFLPPG SILCMTPATS VDTHNICRTS D
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

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 CUG triplet repeat expansion in myotonic dystrophy muscle cells by sequestering the target RNAs.
基因功能參考文獻：
1. Mbnl1(+/-); Mbnl2(+/-) knockout mice with myotonic dystrophy presented with clinical myofibril ultrastructural abnormality and cardiac arrhythmias. PMID: 28416514
2. our study uncovered a novel, autoregulatory function of MBNL proteins based on their binding to e1 of MBNL1 transcript. This function might facilitate cellular protection from MBNL protein level fluctuations, which might otherwise lead to adverse effects caused by extreme MBNL content. PMID: 27903900
3. Data show that Muscleblind-like 1 (Mbnl1) and Muscleblind-Like 3 (Mbnl3) bind skeletal muscle chloride channel CIC-1 (Clc-1) mRNA. PMID: 26501102
4. Depletion of Mbnl1 and/or Mbnl2 reduced localization of hundreds of transcripts, implicating Mbnls in localization of mRNAs to neurites PMID: 26907613
5. Sense DMPK RNA foci clearly co-localize with MBNL1 and MBNL2 proteins and accumulate in myotonic dystrophy 1 tissues during development. PMID: 26339785
6. MBNL1 overexpression promotes transformation of fibroblasts into myofibroblasts. PMID: 26670661
7. These data indicate that MBNL1 plays a conserved role in negatively regulating TGFbeta signaling, and is required for normal valve morphogenesis and homeostasis in vivo. PMID: 26472242
8. this study supports a key role for Mbnl1 loss in the initiation of DM1 cardiac disease. PMID: 25761764
9. Differential expression of Mbnl1 in development plays a role in alternative splicing of vesicular trafficking genes in postnatal heart development. PMID: 24752171
10. Results show that nuclear localization is a major determinant of MBNL1 function. It promotes the nuclear retention of repeat-containing transcripts, which results in repression of aberrant protein expression from the expanded repeats. PMID: 25274774
11. depletion of Mbnl proteins in mouse embryo fibroblasts leads to misregulation of thousands of alternative polyadenylation events. PMID: 25263597
12. consistent with a central and negative regulatory role for MBNL proteins in pluripotency, their knockdown significantly enhances the expression of key pluripotency genes and the formation of induced pluripotent stem cells during somatic cell reprogramming PMID: 23739326
13. MBNL1 overexpression may be a valuable strategy for treating the skeletal muscle features of Myotonic dystrophy . PMID: 22846424
14. This analysis identified several hundred splicing events whose regulation depended on Mbnl function in a pattern indicating functional interchangeability between Mbnl1 and Mbnl2. PMID: 22901804
15. The sequestration of MBNL1 by CUG(exp) RNA results in aberrant splicing events in the Myotonic dystrophy type 1 brain. PMID: 22427994
16. Conserved developmental stage- and tissue-specific alternative splicing of MBNL transcripts is an important mechanism by which MBNL activity is regulated during embryonic development. PMID: 20009516
17. The authors propose that expanded CTG DNA repeats cause two separate effects: loss of Mbnl1 function (disrupting splicing) and loss of another function that disrupts extracellular matrix mRNA regulation, possibly mediated by Mbnl2. PMID: 20098426
18. results show that disruption of the mouse Mbnl1 gene leads to muscle, eye, and RNA splicing abnormalities that are characteristic of myotonic dystrophy disease PMID: 14671308
19. Results support the hypothesis that the loss of muscleblind-like 1 activity is a primary pathogenic event in the development of RNA missplicing and myotonia in myotonic dystrophy. PMID: 16864772
20. CELF and MBNL proteins are determinative for a large subset of splicing transitions that occur during postnatal heart development PMID: 19075228
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亞細胞定位：

Nucleus. Cytoplasm. Cytoplasmic granule.
蛋白家族：

Muscleblind family
組織特異性：

Highly expressed in cardiac and skeletal muscle. Weakly expressed in heart and eye (at protein level).
數據庫鏈接：

KEGG: mmu:56758

STRING: 10090.ENSMUSP00000096686

UniGene: Mm.255723