Silencing mutant ataxin-3 rescues motor deficits and neuropathology in machado-joseph disease transgenic mice.

Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly-inherited neurodegenerative disorder caused by the over-repetition of a CAG codon in the MJD1 gene. This expansion translates into a polyglutamine tract that confers a toxic gain-of-function to the mutant protein - ataxin-3, leading to neurodegeneration in specific brain regions, with particular severity in the cerebellum. No treatment able to modify the disease progression is available. However, gene silencing by RNA interference has shown promising results. Therefore, in this study we investigated whether lentiviral-mediated allele-specific silencing of the mutant ataxin-3 gene, after disease onset, would rescue the motor behavior deficits and neuropathological features in a severely impaired transgenic mouse model of MJD. For this purpose, we injected lentiviral vectors encoding allele-specific silencing-sequences (shAtx3) into the cerebellum of diseased transgenic mice expressing the targeted C-variant of mutant ataxin-3 present in 70% of MJD patients. This variation permits to discriminate between the wild-type and mutant forms, maintaining the normal function of the wild-type allele and silencing only the mutant form. Quantitative analysis of rotarod performance, footprint and activity patterns revealed significant and robust alleviation of gait, balance (average 3-fold increase of rotarod test time), locomotor and exploratory activity impairments in shAtx3-injected mice, as compared to control ones injected with shGFP. An important improvement of neuropathology was also observed, regarding the number of intranuclear inclusions, calbindin and DARPP-32 immunoreactivity, fluorojade B and Golgi staining and molecular and granular layers thickness. These data demonstrate for the first time the efficacy of gene silencing in blocking the MJD-associated motor-behavior and neuropathological abnormalities after the onset of the disease, supporting the use of this strategy for therapy of MJD.

Nomenclatural notes in Membracidae (Homoptera): new combination and new synonymies in Amastris Stål, 1862

The following nomenclatural changes are made: Amastris convoluta (Fabricius, 1781) comb. nov. (formerly Darnis; Hebetica); Amastris maculata Funkhouser, 1922 = Amastris fasciata Broomfield, 1976 syn. nov. = Amastris pseudomaculata Broomfield, 1976 syn. nov. = Amastris inermis Broomfield, 1976 syn. nov. = Amastris sakakibarai Broomfield, 1976 syn. nov.; Amastris elevata Funkhouser, 1922 = Amastris vismiae Haviland, 1925 syn. nov. = Amastris flavifolia Funkhouser, 1927 syn. nov.

Record of two species of Orius Wolff (Hemiptera, Anthocoridae) in Brazil

The genus Orius Wolff, 1811 comprises predatory species, with approximately 70 known species. Informations about the genus in Brazil are scarce. Therefore, the aim of this investigation was to identify Orius species present in four localities in the southeastern Brazil. Samples were taken from several plants, and the material screened in laboratory. The genitalia of both sexes were studied and illustrated. Two species were identified, Orius insidiosus (Say, 1832) and Orius thyestes Herring, 1966. O. insidiosus, the most common species, was collected in all of the localities sampled [Lavras (MG), Holambra, Pindorama and Campinas (SP)]. O. thyestes, registered for the first time in Brazil, occurred only in Lavras (MG) and Pindorama (SP). Some morphologic aspects of these two species are also presented.