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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. dans Nucleic acids research 2014
Mbnl1(+/-); Mbnl2 (Montrer MBNL2 Protéines)(+/-) 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 (Montrer MBNL3 Protéines)) bind skeletal muscle chloride channel (Montrer CLCA1 Protéines) CIC-1 (Clc-1 (Montrer CLCN1 Protéines)) mRNA.
Depletion of Mbnl1 and/or Mbnl2 (Montrer MBNL2 Protéines) reduced localization of hundreds of transcripts, implicating Mbnls in localization of mRNAs to neurites
Sense DMPK (Montrer DMPK Protéines) RNA foci clearly co-localize with MBNL1 and MBNL2 (Montrer MBNL2 Protéines) 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 (Montrer TGFB1 Protéines) 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.
RAN Translation Regulated by Muscleblind Proteins in Myotonic Dystrophy Type 2
Our work suggests that DM1 (Montrer DMPK Protéines) patients are at risk for Fuchs' endothelial corneal dystrophy (FECD (Montrer COL8a2 Protéines)). DMPK (Montrer DMPK Protéines) mutations contribute to the genetic burden of FECD (Montrer COL8a2 Protéines) but are uncommon. We establish a connection between two repeat expansion disorders converging upon RNA-MBNL1 foci and FECD (Montrer COL8a2 Protéines).
Binding of the MBNL zinc fingers to cardiac troponin T (Montrer TNNT2 Protéines) 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 (Montrer HTT Protéines) RNA is mediated by splicing factors MBNL1 and U2AF65 (Montrer U2AF59 Protéines)
muscleblind-like 1 (MBNL1) is a robust suppressor of multiorgan breast cancer metastasis. It binds the 3' untranslated regions of DBNL (Montrer DBNL Protéines) and TACC1 (Montrer TACC1 Protéines) -two genes that are implicated as metastasis suppressors.
abnormal splicing of DMD (Montrer DMD Protéines) exon 78 found in dystrophic muscles of DM1 (Montrer DMPK Protéines) patients is due to the functional loss of MBNL1 and leads to the re-expression of an embryonic dystrophin (Montrer DMD Protéines) in place of the adult isoform.
Reduced RBFOX1 (Montrer A2BP1 Protéines) 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 (Montrer MLXIP Protéines)-1307 leading to low expression of miR (Montrer MLXIP Protéines)-1307-3p in colorectal cancer.
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