CRISPR deletion of the C9ORF72 promoter in ALS/FTD patient motor neurons abolishes production of dipeptide repeat proteins and rescues neurodegeneration
Sign Up to like & getrecommendations! Published in 2020 at "Acta Neuropathologica"
DOI: 10.1007/s00401-020-02154-6
Abstract: GGG GCC (G4C2) repeat expansion in the first intron of C9ORF72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) [6, 11]. Brain tissues from affected individuals show characteristic… read more here.
Keywords: repeat; deletion; c9orf72; poly poly ... See more keywords
Interaction of octahedral ruthenium(II) polypyridyl complex [Ru(bpy)2(PIP)]2+ with poly(U)·poly(A)*poly(U) triplex: Increasing third-strand stabilization of the triplex without affecting the stability of the duplex.
Sign Up to like & getrecommendations! Published in 2017 at "Journal of inorganic biochemistry"
DOI: 10.1016/j.jinorgbio.2017.01.004
Abstract: Triple-helical RNA are of interest because of possible biological roles as well as the potential therapeutic uses of these structures, while the stability of triplexes is usually weaker than that of the Watson-Crick base pairing… read more here.
Keywords: bpy pip; poly triplex; poly; poly poly ... See more keywords
Binding propterties of two Ru(II) polypyridyl complexes containing dppz units and fluorine groups with poly(U)·poly(A) ∗ poly(U) triplex.
Sign Up to like & getrecommendations! Published in 2019 at "Journal of inorganic biochemistry"
DOI: 10.1016/j.jinorgbio.2019.110705
Abstract: In this work, two Ru(II)-dppz (dppz = dipyrido[3,2-a:2',3'-c]phenazine) complexes containing fluorine substituents, [Ru(bpy)2(7-F-dppz)]2+ (Ru1, bpy = 2,2'-bipyridine, 7-F-dppz = 7-fluorodipyrido[3,2-a:2',3'-c]phenazine) and [Ru(phen)2(7-F-dppz)]2+ (Ru2, phen = 1,10-phenanthroline), have been synthesized and characterized. Binding properties of Ru1 and Ru2 with the RNA poly(U)·poly(A) ∗ poly(U) triplex have… read more here.
Keywords: poly poly; poly triplex; dppz; complexes containing ... See more keywords
A phenolic hydroxyl in the ortho- and meta-positions on the main ligands effect on the interactions of [Ru(phen)2(o-HPIP)]2+ and [Ru(phen)2(m-HPIP)]2+ with the poly(U)·poly(A)*poly(U) triplex.
Sign Up to like & getrecommendations! Published in 2020 at "Journal of inorganic biochemistry"
DOI: 10.1016/j.jinorgbio.2020.111268
Abstract: The association of two ruthenium(II) complexes [Ru(phen)2(o-HPIP)]2+ (Ru1; phen = 1,10-phenanthroline, o-HPIP = 2-(2-hydroxyphenyl)-imidazo[4,5-f][1,10] phenanthroline) and [Ru(phen)2(m-HPIP)]2+ (Ru2; m-HPIP = 2-(3-hydroxyphenyl)-imidazo[4,5-f][1,10]phenan- throline) with the RNA poly(U)·poly(A)⁎poly(U) triplex has been investigated by spectrophotometric titrations and melting… read more here.
Keywords: phen hpip; poly triplex; poly; effect ... See more keywords
Extremely high gas permeability of naphthyl group-containing diarylacetylene copolymers
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DOI: 10.1016/j.polymer.2020.123305
Abstract: Abstract: Gas separation using separation membranes is highly advantageous as they consume less energy and are environmentally friendly and inexpensive. Substituted poly(diarylacetylene)s are potential materials for gas-separation membrane owing to the excellent permeation performance. Herein,… read more here.
Keywords: gas; permeability; poly poly; separation ... See more keywords
Binding Differences of Two Homochiral [Ru(bpy)2dppz]2+ Complexes with poly(U)·poly(A)*poly(U) Triplex RNA.
Sign Up to like & getrecommendations! Published in 2017 at "Inorganic chemistry"
DOI: 10.1021/acs.inorgchem.7b00670
Abstract: The first investigation of chiral ruthenium(II) complexes Δ- and Λ-[Ru(bpy)2dppz]2+ and triplex RNA poly(U)·poly(A)*poly(U) was carried out, which showed that Δ enantiomer displayed significant ability in stabilizing model triplex RNA. read more here.
Keywords: bpy 2dppz; poly; poly poly; binding differences ... See more keywords
Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Neurodegeneration"
DOI: 10.1186/s13024-019-0310-z
Abstract: BackgroundA G4C2 hexanucleotide repeat expansion in the noncoding region of C9orf72 is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). Putative disease mechanisms underlying c9FTD/ALS include toxicity from sense G4C2 and… read more here.
Keywords: stress granule; deposition; poly poly; stress ... See more keywords