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anti-Mouse (Murine) MBNL1 Antikörper:
anti-Human MBNL1 Antikörper:
anti-Rat (Rattus) MBNL1 Antikörper:
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Human Polyclonal MBNL1 Primary Antibody für ELISA, WB - ABIN451638
Warf, Berglund: MBNL binds similar RNA structures in the CUG repeats of myotonic dystrophy and its pre-mRNA substrate cardiac troponin T. in RNA (New York, N.Y.) 2007
Human Polyclonal MBNL1 Primary Antibody für IHC, WB - ABIN2776698
Adereth, Dammai, Kose, Li, Hsu: RNA-dependent integrin alpha3 protein localization regulated by the Muscleblind-like protein MLP1. in Nature cell biology 2005
Show all 3 Pubmed References
Dog (Canine) Polyclonal MBNL1 Primary Antibody für IHC, WB - ABIN2776697
Coram, Stillwagon, Guggilam, Jenkins, Swanson, Ladd: Muscleblind-like 1 is required for normal heart valve development in vivo. in BMC developmental biology 2015
Human Monoclonal MBNL1 Primary Antibody für IF, IHC (p) - ABIN561753
Rehman, Gladman, Periasamy, Sun, Mahadevan: Development of an AP-FRET based analysis for characterizing RNA-protein interactions in myotonic dystrophy (DM1). in PLoS ONE 2014
MBNL1 cytoplasmic, but not nuclear, isoform promotes neurite morphogenesis and reverses the morphological defects caused by expanded CUG RNA.
This study uncovers non-canonical functions of MBNL proteins (Mbnl1/2) that bind and promote the expression of miR-294 targets, including Cdkn1a and Tgfbr2, thereby opposing the role of miR-294 in regulating cell proliferation, apoptosis, and epithelial-mesenchymal transition (EMT).
Mbnl1(+/-); Mbnl2(+/-) knockout mice with myotonic dystrophy presented with clinical myofibril ultrastructural abnormality and cardiac arrhythmias.
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.
Data show that Muscleblind-like 1 (Mbnl1) and Muscleblind-Like 3 (Mbnl3) bind skeletal muscle chloride channel CIC-1 (Clc-1) mRNA.
Depletion of Mbnl1 and/or Mbnl2 reduced localization of hundreds of transcripts, implicating Mbnls in localization of mRNAs to neurites
Sense DMPK RNA foci clearly co-localize with MBNL1 and MBNL2 proteins and accumulate in myotonic dystrophy 1 tissues during development.
MBNL1 overexpression promotes transformation of fibroblasts into myofibroblasts.
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.
this study supports a key role for Mbnl1 loss in the initiation of DM1 cardiac disease.
Differential expression of Mbnl1 in development plays a role in alternative splicing of vesicular trafficking genes in postnatal heart development.
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.
depletion of Mbnl proteins in mouse embryo fibroblasts leads to misregulation of thousands of alternative polyadenylation events.
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
MBNL1 overexpression may be a valuable strategy for treating the skeletal muscle features of Myotonic dystrophy .
This analysis identified several hundred splicing events whose regulation depended on Mbnl function in a pattern indicating functional interchangeability between Mbnl1 and Mbnl2.
The sequestration of MBNL1 by CUG(exp) RNA results in aberrant splicing events in the Myotonic dystrophy type 1 brain.
Muscleblind1, but not Dmpk or Six5, contributes to a complex phenotype of muscular and motivational deficits in mouse models of myotonic dystrophy
Conserved developmental stage- and tissue-specific alternative splicing of MBNL transcripts is an important mechanism by which MBNL activity is regulated during embryonic development.
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.
MBNL1 promotes cell differentiation through regulation of alternative splicing, and we demonstrate that TRIM71 promotes embryonic splicing patterns through MBNL1 repression
MBNL1 overexpression altered cerebral cortical responsiveness by decreasing the response in the contralateral hemisphere evoked by electrical stimulation.
The structure of targeted RNAs is a prevalent component of the mechanism of alternative splicing regulation by MBNLs.
MBNL1 is an important alternative splicing regulator during differentiation of monocytes.
The knockdown of muscleblindlike 1 (MBNL1) induces epithelial-to-mesenchymal transition (EMT)-like morphological changes in the colorectal cancer (CRC) HCT-116 cells, accompanied by an enhanced cell motility, and by the downregulation of E-cadherin and the upregulation of Snail expression. Overexpression of MBNL1 suppresses EMT, characterized by the upregulation of Ecadherin and the downregulation of Snail expression.
RBFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences.
Functional depletion of the alternative splicing factors Muscleblind-like (MBNL 1 and 2) is at the basis of the neuromuscular disease myotonic dystrophy type 1 (DM1). Here, we screen for miRNAs that regulate MBNL1 and MBNL2 in HeLa cells. We thus identify miR-23b and miR-218, and confirm that they downregulate MBNL proteins in this cell line.
For exogenous activation of MBNL transcription, MBNL1 transcription start site T2 seems to be the most suitable target, as the ensuing pre-mRNA is susceptible to both major loops, e1 and e5, and hence, theoretically, following induction each cell in the body could reach the optimal MBNL content.
RAN Translation Regulated by Muscleblind Proteins in Myotonic Dystrophy Type 2
Our work suggests that DM1 patients are at risk for Fuchs' endothelial corneal dystrophy (FECD). DMPK mutations contribute to the genetic burden of FECD but are uncommon. We establish a connection between two repeat expansion disorders converging upon RNA-MBNL1 foci and FECD.
Binding of the MBNL zinc fingers to cardiac troponin T pre-mRNA is specific and relatively simple, unlike the complex multiple dimer-trimer stoichiometries postulated in some previous studies.
Heterozygous missense mutations and one in-frame deletion in MBNL1 were identified in 3 myotonic dystrophy patients.
Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65
muscleblind-like 1 (MBNL1) is a robust suppressor of multiorgan breast cancer metastasis. It binds the 3' untranslated regions of DBNL and TACC1 -two genes that are implicated as metastasis suppressors.
abnormal splicing of DMD exon 78 found in dystrophic muscles of DM1 patients is due to the functional loss of MBNL1 and leads to the re-expression of an embryonic dystrophin in place of the adult isoform.
Reduced RBFOX1 activity in myotonic dystrophy type 1 tissues may amplify several of the splicing alterations caused by the deficiency in MBNL1.
MBNL1 binds with C allelic pre-miR-1307 leading to low expression of miR-1307-3p in colorectal cancer.
The result is consistent with the hypothesis that MBNL proteins are trapped by expanded CUG repeats and inactivated in myotonic dystrophy type 1 (DM1) and that CELF1 is activated in DM1.
Involved in pre-mRNA alternative splicing regulation. Binds to CUG triplet repeat in RNA (By similarity).
, muscleblind-like (Drosophila)
, muscleblind-like protein 1
, muscleblind-like 1
, triplet-expansion RNA-binding protein