CAG repeat length correlated inversely with age of onset of visual or motor signs (r = -0.74, p = 0.002). Stage 3 rate of progression did not differ between cases (p = 0.18). Electroretinograms correlated with Brief Ataxia Rating Scale score and were a biomarker AC220 of disease onset and progression. All symptomatic patients developed gait ataxia, extremity dysmetria, dysarthria, dysrhythmia, and oculomotor abnormalities. Funduscopy revealed pale optic discs and pigmentary disturbances. Visual acuity declined to blindness in those with longer CAG
expansions. Hyperreflexia was present from Stage 1 onwards. Restless legs syndrome and sensory impairment were common. Neuropathological hallmarks were neuronal loss in cerebellar cortex, deep cerebellar nuclei, inferior olive, and anterior horns of the spinal cord, and axonal loss in spinocerebellar
tracts, dorsal nerve roots, and posterior columns. Retinal pathology included photoreceptor degeneration and disruption of retinal pigment epithelium. Spinocerebellar ataxia type 7 evolves through four clinical stages; neuropathological findings underlie the clinical presentation; electroretinograms are a potential biomarker of disease progression.”
“Chemical investigation of the leaves of Ilex hainanensis Merr. yielded a new flavanone named Huazhongilexone-7-O–D-glucopyranoside, KU-57788 mw together with five known compounds, which included caffeic acid, quercetin, isoquercitrin, quercitrin-7-O–D-glucopyranoside and rutin. All of the compounds were isolated from the plant for the first time. The structure of the new compound was determined on the basis of high resolution mass, 1D and 2D NMR spectroscopies including ROESY. The antioxidant activity of the new compound was assessed using the DPPH spectrophotometric and IC50 value was 10.39 mol L-1.”
“Phytochemical study of the methanol extract of Artocarpus altilis resulted in the isolation of a new prenylated aurone, artocarpaurone (1),
together with eight known compounds including two prenylated chalcones (2 and 3), three prenylated flavanones (4-6), and three triterpenes (7-9). The structure of 1 was elucidated MEK activation as 6-hydroxy-2-[8-hydroxy-2-methyl-2-(4-methyl-3-pentenyl)-2H-1-benzopyran-5-ylmethylene]-3(2H)-benzofuranone by spectroscopic methods including 1D and 2D NMR spectra and FT-ICR-MS. Compound 1 showed moderate nitric oxide radical scavenging activity, whereas 2 and 3 had moderate 2,2-diphenyl-1-picrylhydrazyl radical scavenging effect, compared with the positive control (+)-catechin.”
“We developed a method for deep mutational scanning of antibody complementarity-determining regions (CDRs) that can determine in parallel the effect of every possible single amino acid CDR substitution on antigen binding.