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Human MBNL1 Protein expressed in HEK-293 Cells - ABIN2725584
Echeverria, Cooper: Muscleblind-like 1 activates insulin receptor exon 11 inclusion by enhancing U2AF65 binding and splicing of the upstream intron. in Nucleic acids research 2014
Mbnl1(+/-); Mbnl2 (zeige MBNL2 Proteine)(+/-) 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 (zeige MBNL3 Proteine)) bind skeletal muscle chloride channel (zeige CLCA1 Proteine) CIC-1 (Clc-1 (zeige CLCN1 Proteine)) mRNA.
Depletion of Mbnl1 and/or Mbnl2 (zeige MBNL2 Proteine) reduced localization of hundreds of transcripts, implicating Mbnls in localization of mRNAs to neurites
Sense DMPK (zeige DMPK Proteine) RNA foci clearly co-localize with MBNL1 and MBNL2 (zeige MBNL2 Proteine) 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 (zeige TGFB1 Proteine) 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.
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 (zeige DMPK Proteine) patients are at risk for Fuchs' endothelial corneal dystrophy (FECD (zeige COL8a2 Proteine)). DMPK (zeige DMPK Proteine) mutations contribute to the genetic burden of FECD (zeige COL8a2 Proteine) but are uncommon. We establish a connection between two repeat expansion disorders converging upon RNA-MBNL1 foci and FECD (zeige COL8a2 Proteine).
Binding of the MBNL zinc fingers to cardiac troponin T (zeige TNNT2 Proteine) 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 (zeige HTT Proteine) RNA is mediated by splicing factors MBNL1 and U2AF65 (zeige U2AF59 Proteine)
muscleblind-like 1 (MBNL1) is a robust suppressor of multiorgan breast cancer metastasis. It binds the 3' untranslated regions of DBNL (zeige DBNL Proteine) and TACC1 (zeige TACC1 Proteine) -two genes that are implicated as metastasis suppressors.
abnormal splicing of DMD (zeige DMD Proteine) exon 78 found in dystrophic muscles of DM1 (zeige DMPK Proteine) patients is due to the functional loss of MBNL1 and leads to the re-expression of an embryonic dystrophin (zeige DMD Proteine) in place of the adult isoform.
Reduced RBFOX1 (zeige A2BP1 Proteine) activity in myotonic dystrophy type 1 tissues may amplify several of the splicing alterations caused by the deficiency in MBNL1.
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