ATP1A3 mutation as a candidate cause of autosomal dominant cone-rod dystrophy.

Cone-rod dystrophy (CORD) is an inherited retinal degenerative disease characterized by progressive loss of cone and rod photoreceptors. Although several genes have been reported to cause autosomal dominant CORD (adCORD), the genetic causes of adCORD have not been fully elucidated. Here, we identified the ATP1A3 gene, encoding the α3 subunit of Na, K-ATPase, as a novel gene associated with adCORD.

Deletion of miR-182 Leads to Retinal Dysfunction in Mice.

Purpose: MicroRNA-182 (miR-182) is abundantly expressed in mammalian retinas; however, the association between miR-182 and retinal function remains unclear. In this study, we explored whether miR-182 contributes to functional decline in retinas using a miR-182 depleted mouse.

Methods: Electroretinogram (ERG) amplitudes at different ages were measured in miR-182 knockout (KO) mice.

miR-183/96 plays a pivotal regulatory role in mouse photoreceptor maturation and maintenance.

MicroRNAs (miRNAs) are known to be essential for retinal maturation and functionality; however, the role of the most abundant miRNAs, the miR-183/96/182 cluster (miR-183 cluster), in photoreceptor cells remains unclear. Here we demonstrate that ablation of two components of the miR-183 cluster, miR-183 and miR-96, significantly affects photoreceptor maturation and maintenance in mice. Morphologically, early-onset dislocated cone nuclei, shortened outer segments and thinned outer nuclear layers are observed in the miR-183/96 double-knockout (DKO) mice.

Targeted RP9 ablation and mutagenesis in mouse photoreceptor cells by CRISPR-Cas9.

Precursor messenger RNA (Pre-mRNA) splicing is an essential biological process in eukaryotic cells. Genetic mutations in many spliceosome genes confer human eye diseases. Mutations in the pre-mRNA splicing factor, RP9 (also known as PAP1), predispose autosomal dominant retinitis pigmentosa (adRP) with an early onset and severe vision loss.

Toll-Like Receptor 3 Activation Initiates Photoreceptor Cell Death In Vivo and In Vitro.

Purpose: Accumulating evidence has demonstrated that excessive immunoreaction plays a prominent role in the pathogenesis of dry AMD. Toll-like receptor 3 (TLR3) can be activated by double-stranded (ds)RNA in retinal pigment epithelia and trigger an innate immunity-mediated inflammatory response. However, its role in photoreceptor cells, the effectors of AMD geographic atrophy, remains unclear.

Novel fluorescent cephalosporins: synthesis, antimicrobial activity and photodynamic inactivation of antibiotic resistant bacteria.

Two novel fluorescent cephalosporins, TCA and TBCA, were synthesized and characterized by (1)H NMR, (13)C NMR, UV-vis, and fluorescence spectroscopies. Biological activity assays demonstrated that TCA inactivated a Klebsiella pneumonia strain that expressed extended-spectrum β-lactamases. Incubation of 6 μM TCA with K.

Synthesis and activity study of phosphonamidate dipeptides as potential inhibitors of VanX.

In an effort to develop inhibitors of VanX, the phosphonamidate analogs of D-Ala-D-Ala dipeptides, N-[(1-aminoethyl) hydroxyphosphinyl]-glycine (1a), -alanine (1b), -valine (1c), -leucine (1d) and -phenylalanine (1e) were synthesized, characterized and evaluated using recombinant VanX. The crystal structure of the intermediate 6d was obtained (Deposition number: CCDC 839134), and structural analysis revealed that it is orthorhombic with a space group P2(1)2(1)2(1), the bond length of P-N is 1.62Å and angle of C-N-P is 123.

Novel conjugation of norvancomycin-fluorescein for photodynamic inactivation of Bacillus subtilis.

A simple and unique conjugation of norvancomycin-fluorescein (VanF) has been achieved. It was characterized by UV-vis and fluorescence spectra and confirmed by MALDI-TOF mass spectrum. The photodynamic assay indicated that VanF effectively inactivated the Gram-positive Bacillus subtilis (ATCC 6633) from clinic with inactivation rate of 30-70% within 1-7.