Abstract：
:The Duchenne muscular dystrophy (DMD) gene has a complex expression pattern regulated by multiple tissue-specific promoters and by alternative splicing (AS) of the resulting transcripts. Here, we used an RNAi-based approach coupled with DMD-targeted RNA-seq to identify RNA-binding proteins (RBPs) that regulate splicing of its skeletal muscle isoform (Dp427m) in a human muscular cell line. A total of 16 RBPs comprising the major regulators of muscle-specific splicing events were tested. We show that distinct combinations of RBPs maintain the correct inclusion in the Dp427m of exons that undergo spatio-temporal AS in other dystrophin isoforms. In particular, our findings revealed the complex networks of RBPs contributing to the splicing of the two short DMD exons 71 and 78, the inclusion of exon 78 in the adult Dp427m isoform being crucial for muscle function. Among the RBPs tested, QKI and DDX5/DDX17 proteins are important determinants of DMD exon inclusion. This is the first large-scale study to determine which RBP proteins act on the physiological splicing of the DMD gene. Our data shed light on molecular mechanisms contributing to the expression of the different dystrophin isoforms, which could be influenced by a change in the function or expression level of the identified RBPs.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Miro J,Bougé AL,Murauer E,Beyne E,Da Cunha D,Claustres M,Koenig M,Tuffery-Giraud Sdoi
10.3390/ijms21207803subject
Has Abstractpub_date
2020-10-21 00:00:00issue
20issn
1422-0067pii
ijms21207803journal_volume
21pub_type
杂志文章abstract：:To design ARC-111 analogues with improved efficiency, we constructed the QSAR of 22 ARC-111 analogues with RPMI8402 tumor cells. First, the optimized support vector regression (SVR) model based on the literature descriptors and the worst descriptor elimination multi-roundly (WDEM) method had similar generalization as ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms13011161
更新日期：2012-01-01 00:00:00
abstract：:Protein kinase is a novel therapeutic target for human diseases. The off-target and side effects of ATP-competitive inhibitors preclude them from the clinically relevant drugs. The compounds targeting the druggable allosteric sites outside the highly conversed ATP binding pocket have been identified as promising alter...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19010111
更新日期：2018-01-01 00:00:00
abstract：:This pilot study aims to investigate whether salivary small extracellular vesicle (sEV)-associated microRNAs could act as potential biomarkers for periodontal disease status. Twenty-nine participants (10 who were healthy, nine with gingivitis, 10 with stage III/IV periodontitis) were recruited and unstimulated whole s...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21082809
更新日期：2020-04-17 00:00:00
abstract：:InPBi exhibits broad and strong photoluminescence at room temperature, and is a potential candidate for fabricating super-luminescence diodes applied in optical coherence tomography. In this paper, the strained InPBi quantum dot (QD) embedded in the AlGaAs barrier on a GaAs platform is proposed to enhance the light em...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms20236001
更新日期：2019-11-28 00:00:00
abstract：:Sex reversal has been used as a breeding strategy by salmonid fish to produce genetically and phenotypically single sex populations. Production of all-female fish has great importance for the creation of monosex female triploids of salmonid fish, which are valued for their sterility, lack of female maturation, and lar...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms22020964
更新日期：2021-01-19 00:00:00
abstract：:The present study was designed to probe the effects of Huperzine A (HupA) on diabetes-associated cognitive decline (DACD) using a streptozotocin (STZ)-injected rat model. Diabetic rats were treated with HupA (0.05 and 0.1 mg/kg) for seven weeks. Memory functions were evaluated by the water maze test. Nissl staining wa...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms15057667
更新日期：2014-05-05 00:00:00
abstract：:Vascular inflammation plays a key role in the pathogenesis and progression of atherosclerosis, a main complication of diabetes. The present study investigated whether an aqueous extract of Portulaca oleracea (AP) prevents the TNF-α-induced vascular inflammatory process in the human umbilical vein endothelial cell (HUV...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms13055628
更新日期：2012-01-01 00:00:00
abstract：:Liver fibrosis is a complex process associated to most types of chronic liver disease, which is characterized by a disturbance of hepatic tissue architecture and the excessive accumulation of extracellular matrix. Resolvin E1 (RvE1) is a representative member of the eicosapentaenoic omega-3 lipid derivatives, and is a...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21228827
更新日期：2020-11-22 00:00:00
abstract：:Gene fusions have a very important role in the study of cancer development. In this regard, predicting the probability of protein fusion transcripts of developing into a cancer is a very challenging and yet not fully explored research problem. To this date, all the available approaches in literature try to explain the...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms20071645
更新日期：2019-04-02 00:00:00
abstract：:To investigate the effect of pollination on the fruit quality of 'Dangshan Su' pear, 'Dangshan Su' was fertilized by the pollen of 'Wonhwang' (Pyrus pyrifolia Nakai.) (DW) and 'Jingbaili' (Pyrus ussuriensis Maxim.) (DJ). The analysis of primary metabolites was achieved through untargeted metabolomics, and the quantita...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19082273
更新日期：2018-08-02 00:00:00
abstract：:Connexins are the structural components of gap junctions and hemichannels that mediate the communication and exchange of small molecules between cells, and between the intracellular and extracellular environment, respectively. Connexin (Cx) 46 is predominately expressed in lens fiber cells, where they function in main...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms21218194
更新日期：2020-11-02 00:00:00
abstract：:Zinc oxide nanoparticles (ZnO NPs) have shown adverse health impact on the human male reproductive system, with evidence of inducing apoptosis. However, whether or not ZnO NPs could promote autophagy, and the possible role of autophagy in the progress of apoptosis, remain unclear. In the current study, in vitro and in...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms20164042
更新日期：2019-08-19 00:00:00
abstract：:Ionic liquids (ILs) are chemical compounds composed of ions with melting points below 100 °C exhibiting a design feature. ILs are commonly used as the so-called green solvents, reagents or highly efficient catalysts in varied chemical processes. The huge application potential of ionic liquids (IL) justifies the growin...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms21124253
更新日期：2020-06-15 00:00:00
abstract：:Mitochondria are involved either directly or indirectly in oncogenesis and the alteration of metabolism in cancer cells. Cancer cells contain large numbers of abnormal mitochondria and produce large amounts of reactive oxygen species (ROS). Oxidative stress is caused by an imbalance between the production of ROS and t...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms12107163
更新日期：2011-01-01 00:00:00
abstract：:Alzheimer's disease (AD) is a common neurodegenerative disease in elderly individuals, and effective therapies are unavailable. This study was designed to investigate the neuroprotective effects of sulforaphane (an activator of NF-E2-related factor 2) on mice with AD-like lesions induced by combined administration of ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms150814396
更新日期：2014-08-18 00:00:00
abstract：:Understanding the kinetics of peptide self-assembly is important because of the involvement of peptide amyloid fibrils in several neurodegenerative diseases. In this paper, we have studied the dissolution kinetics of self-assembled model peptide fibrils after a dilution quench. Due to the low concentrations involved, ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21207671
更新日期：2020-10-16 00:00:00
abstract：:Chimeric antigen receptors (CARs) have found clinical success in B cell malignancies, but a dearth of potential targets limits their wider clinical application, especially in solid tumours. Here, we describe the development of an anti-annexin A2 CAR, CAR(2448), derived from an antibody found to have activity against e...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21020381
更新日期：2020-01-07 00:00:00
abstract：:Glioblastoma (GBM) is the most common malignant brain tumor and its malignant phenotypic characteristics are classified as grade IV tumors. Molecular interactions, such as protein-protein, protein-ncRNA, and protein-peptide interactions are crucial to transfer the signaling communications in cellular signaling pathway...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms22020517
更新日期：2021-01-06 00:00:00
abstract：:Diabetic complications are the major cause of mortality for the patients with diabetes. Oxidative stress and inflammation have been recognized as important contributors for the development of many diabetic complications, such as diabetic nephropathy, hepatopathy, cardiomyopathy, and other cardiovascular diseases. Seve...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms17101629
更新日期：2016-09-24 00:00:00
abstract：:Antimicrobial peptides have been identified as one of the alternatives to the extensive use of common antibiotics as they show a broad spectrum of activity against human pathogens. Among these is Chionodracine (Cnd), a host-defense peptide isolated from the Antarctic icefish Chionodracohamatus, which belongs to the fa...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21041401
更新日期：2020-02-19 00:00:00
abstract：:Nut-based milks and yogurts are gaining popularity, but may not offer the same benefits as dairy yogurts to consumers. Cashew nuts often cause severe allergic reactions, and cashew nut allergens are stable to several types of processing. To compare its characteristics to dairy yogurt and characterize the effects of fe...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21218267
更新日期：2020-11-04 00:00:00
abstract：:This review analyzes current studies of the therapeutic effects of Phoenix dactylifera, or date palm fruit, on the physiologic system. Specifically, we sought to summarize the effects of its application in preventing cell damage, improving cancer therapeutics and reducing damage caused by conventional chemotherapy. Ph...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms161226210
更新日期：2015-12-17 00:00:00
abstract：:Magnetic resonance spectroscopy (MRS) is a non-invasive method for quantitative estimation of liver fat. Knowledge of its imprecision, which comprises biological variability and measurement error, is required to design therapeutic trials with measurement of change. The role of adipocyte lipolysis in ectopic fat accumu...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17071089
更新日期：2016-07-08 00:00:00
abstract：:In general, a drug candidate is evaluated using 2D-cultured cancer cells followed by an animal model. Despite successful preclinical testing, however, most drugs that enter human clinical trials fail. The high failure rates are mainly caused by incompatibility between the responses of the current models and humans. He...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18112348
更新日期：2017-11-07 00:00:00
abstract：:Vulvar squamous cell carcinoma (VSCC) originates from the progression of either a high-grade squamous intraepithelial lesion (HSIL) or differentiated-type vulvar intraepithelial neoplasia (dVIN), often in a background of lichen sclerosus (LS). The mechanisms leading to the progression of these premalignant lesions to ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21144880
更新日期：2020-07-10 00:00:00
abstract：:Sulfur (S) is an essential element for all forms of life. It is involved in numerous essential processes because S is considered as the primary source of one of the essential amino acids, methionine, which plays an important role in biological events. For the control and regulation of sulfate in a metabolic network th...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21072572
更新日期：2020-04-07 00:00:00
abstract：:Carnitine has vital roles in the endogenous metabolism of short chain fatty acids. It can protect and support gut microbial species, and some dietary fibers can reduce the available iron involved in the bioactivity of carnitine. There is also an antagonistic relationship between high microbial populations and carnitin...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms19041008
更新日期：2018-03-28 00:00:00
abstract：:Chemical injury is partly due to free radical lipid peroxidation, which can induce oxidative stress and produce a large number of reactive oxygen species (ROS). Oxaloacetic acid is an important intermediary in the tricarboxylic acid cycle (TCA cycle) and participates in metabolism and energy production. In our study, ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19061626
更新日期：2018-05-31 00:00:00
abstract：:It has been suggested that GAP-43 (growth-associated protein) binds to various proteins in growing neurons as part of its mechanism of action. To test this hypothesis in vivo, differentiated N1E-115 neuroblastoma cells were labeled with [(35)S]-amino acids and were treated with a cleavable crosslinking reagent. The ce...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms9091753
更新日期：2008-09-01 00:00:00
abstract：:Under acidic conditions and at high ionic strength, the zinc cation is removed from its metal complex with 5,10,15,20-tetrakis(4-sulfonatophenyl)porphyrin (TPPS4) thus leading to the diacid free porphyrin, that subsequently self-organize into J-aggregates. The kinetics of the demetallation step and the successive supr...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21114001
更新日期：2020-06-03 00:00:00
