about
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionLong non-coding RNAs in nervous system function and diseaseSequencing the unsequenceable: expanded CGG-repeat alleles of the fragile X geneA natural antisense transcript at the Huntington's disease repeat locus regulates HTT expressionCoordinate regulation of mRNA decay networks by GU-rich elements and CELF1Steroid receptor RNA activator protein binds to and counteracts SRA RNA-mediated activation of MyoD and muscle differentiationEpigenetics and Triplet-Repeat Neurological DiseasesConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisSpecific and Modular Binding Code for Cytosine Recognition in Pumilio/FBF (PUF) RNA-binding DomainsUtilizing the GAAA Tetraloop/Receptor To Facilitate Crystal Packing and Determination of the Structure of a CUG RNA HelixMolecular genetics and genetic testing in myotonic dystrophy type 1.Yeast Gis2 and its human ortholog CNBP are novel components of stress-induced RNP granules.Welander distal myopathy is caused by a mutation in the RNA-binding protein TIA1Engineering of an elastic scaffolding polyprotein based on an SH3-binding intrinsically disordered titin PEVK module.Hypogonadism Associated with Cyp19a1 (Aromatase) Posttranscriptional Upregulation in Celf1 Knockout Mice.The RNA-binding protein Staufen1 is increased in DM1 skeletal muscle and promotes alternative pre-mRNA splicingComputational investigation of RNA CUG repeats responsible for myotonic dystrophy 1Myotonic dystrophy type 1 patient-derived iPSCs for the investigation of CTG repeat instability.Advances in understanding the molecular basis of FXTASThe four Zn fingers of MBNL1 provide a flexible platform for recognition of its RNA binding elements.Directed PCR-free engineering of highly repetitive DNA sequences.Myoblasts generated by lentiviral mediated MyoD transduction of myotonic dystrophy type 1 (DM1) fibroblasts can be used for assays of therapeutic molecules.A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity.Fragile X and autism: Intertwined at the molecular level leading to targeted treatments.RNA therapeutics: beyond RNA interference and antisense oligonucleotides.RNase H-mediated degradation of toxic RNA in myotonic dystrophy type 1The emerging roles of microRNAs in the pathogenesis of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disordersModifications to toxic CUG RNAs induce structural stability, rescue mis-splicing in a myotonic dystrophy cell model and reduce toxicity in a myotonic dystrophy zebrafish model.RNA structure replaces the need for U2AF2 in splicing.Analysis of in situ pre-mRNA targets of human splicing factor SF1 reveals a function in alternative splicingSmaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy.The role of CELF proteins in neurological disorders.Clinical ramifications of the MHC family Fc receptor FcRn.Alternative splicing dysregulation secondary to skeletal muscle regeneration.Analysis of exonic regions involved in nuclear localization, splicing activity, and dimerization of Muscleblind-like-1 isoformsDeveloping bivalent ligands to target CUG triplet repeats, the causative agent of myotonic dystrophy type 1.Massive expansion of SCA2 with autonomic dysfunction, retinitis pigmentosa, and infantile spasmsAutoregulated splicing of muscleblind-like 1 (MBNL1) Pre-mRNA.Global assessment of GU-rich regulatory content and function in the human transcriptomeTransgenic mice with SCA10 pentanucleotide repeats show motor phenotype and susceptibility to seizure: a toxic RNA gain-of-function model.
P2860
Q24562489-9ED7EA5B-E4E4-4E43-9BE5-9CD38E838092Q24595656-7365A0EE-DF26-4F53-938F-B19F13EF530AQ24596776-A09909F1-DFA0-43D4-AE31-99D80EE04341Q24620388-BACA9459-A33D-4DAC-9951-B1D063AB8F90Q24626214-A190B510-4667-4DE8-A233-7B707A9686B3Q24633963-70074ED7-CFD5-47A9-9D9F-993EED7A2AACQ26770705-622AB3CD-FDA5-4A2B-A0AE-3B99E870A77DQ26863439-A7CBEB0C-86F9-4A7C-AA26-5E4B5B705230Q27666276-50844330-ED1E-4C11-8FCA-8A975CF814BFQ27673782-CE749B9D-3C7C-49F8-92C4-5DDD2F74634DQ27691417-568B7651-CDC8-4FC2-8DBB-3A0AD43A66DBQ27937013-6D23B803-03A4-4477-AB69-1AE1E7C68E93Q28285385-F5708FDB-F371-435D-9DE7-F29DD7197A9CQ30010028-BC9A6E3D-EC04-4894-88A3-DA2673C908D6Q30360551-8EDD8E10-D744-448F-B756-7972A8A13CA3Q30424920-978512E9-BB00-4AEB-919B-12012F175F3AQ30668605-97BF9210-AF31-4DE1-B41D-0BF63A5C309CQ30838313-4B1EEDC9-0674-4048-91EF-37700062BEAEQ33871325-4A269DB3-E5D2-4B67-A4CC-7CA45CE64059Q33891599-75DBD909-D1C0-4187-AF5B-AC9CFB12D96DQ34029622-1B150EE6-A1AE-4F9C-BC6A-2B8282CA0C8FQ34073069-E10762E0-5355-44B3-9EDA-F23A3C6D7501Q34181368-99C59F58-A3D5-4421-AAC8-EF629BB03C36Q34198650-76CD6CDC-F765-4E9B-94D3-EF06FCAEB38EQ34248493-81EDE3C5-6AD0-4C55-BFAE-1CB4CFD78658Q34257340-555C828A-912E-4E8D-89E5-13E6164319F3Q34353055-6938E039-A912-4514-8727-6F6FE33B36FAQ34489742-77A7A870-2386-4406-80B6-F15C6CBB60A0Q34501702-8FCBAE57-391D-497E-A40C-A390D172704EQ34699766-C05106E5-62CC-4146-8651-1FDDF982218EQ34699771-C64A77CC-A74F-44A4-8D02-2AD10D48A9F4Q34706897-87999BD1-673E-4959-8FBB-A44B27CDF3C5Q34754673-71068B33-3A85-41F3-8AD5-A44278AA73C3Q34861423-FCA117D0-C5A7-4485-9430-6C86445EE315Q34963754-77AA9BA6-8DEF-4410-A5E6-A0C3CEFEB789Q35031314-B73094F7-FCC8-41D6-981F-42DFE5987D60Q35215622-20E2BCCF-8237-44C0-8BB9-BFAAE0D0A28FQ35310396-570C27F4-05BE-4450-A236-273411D3D333Q35578540-8FB21849-A693-457D-8F09-9261277BD26BQ35839528-DBB9E30E-25C1-411D-8B6E-07FEC82E4480
P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on December 2009
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Pathogenic mechanisms of myotonic dystrophy
@en
Pathogenic mechanisms of myotonic dystrophy.
@nl
type
label
Pathogenic mechanisms of myotonic dystrophy
@en
Pathogenic mechanisms of myotonic dystrophy.
@nl
prefLabel
Pathogenic mechanisms of myotonic dystrophy
@en
Pathogenic mechanisms of myotonic dystrophy.
@nl
P2860
P356
P1476
Pathogenic mechanisms of myotonic dystrophy
@en
P2093
Johanna E Lee
Thomas A Cooper
P2860
P304
P356
10.1042/BST0371281
P577
2009-12-01T00:00:00Z