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.

O gênero Callideriphus Blanchard, 1851 (Coleoptera, Cerambycidae, Heteropsini)

The genus Callideriphus Blanchard, 1851 (Coleoptera, Cerambycidae, Heteropsini). The genus Callideriphus comprises only two species: C. grossipes Blanchard, 1851 (type species) and C. tucumanus sp. nov. (Argentina, Tucumán). The type locality of C. grossipes had been originally indicated as Chile, but it is supposedly considered erroneous. Its distribution, actually, extends from Southeastern Brazil up to Argentina, along the Atlantic Forest. This species is extremely variable in regard to its coloration and elytral punctation. Two subspecies are recognized: C. grossipes grossipes Blanchard, 1851 (BRAZIL: Minas Gerais, Espírito Santo, São Paulo, Paraná, Santa Catarina) and C. grossipes flavipennis Melzer, 1934 (BRAZIL: Santa Catarina, Rio Grande do Sul; ARGENTINA: Chaco, Entre Ríos, Buenos Aires). Four intermediate forms are recorded and commented. Redescription and a key to species are added. Nomenclatural changes: Callideriphus grossipes grossipes Blanchard, 1851 = C. grossipes var. brasliensis Melzer, 1923 syn. nov. = C. rubricollis Melzer, 1934 syn. nov.; Callideriphus grossipes flavipennis Melzer, 1934 stat nov. = C. signaticollis Melzer, 1934 syn. nov.

Revisão do gênero Mecocephala Dallas, 1851 (Heteroptera, Pentatomidae)

Revision of the genus Mecocephala Dallas, 1851 (Heteroptera, Pentatomidae). The genus Mecocephala Dallas, 1851 is revised, and some taxonomic and geographical distribution data are evaluated. The following species are considered to belong to this genus: M. acuminata Dallas, 1851 = M. holmbergi Pirán, 1969 syn. nov., M. curculionoides Pirán, 1959, M. bonariensis sp. nov., M. magna sp. nov., M. maldonadensis sp. nov., and M. zikani sp. nov.; their distribution is restricted to southern Neotropical Region. Other species, formerly placed in Mecocephala, are considered, respectively: M. rubripes Berg, 1894 incertae sedis, M. darwini Kirkaldy, 1909 incertae sedis, M. atra Bergroth, 1914 incertae sedis, Paramecocephala uruguayensis (Pirán, 1970) comb. nov., Paramecocephala fusca (Haglund, 1868) comb. nov. A key to the species is presented.

Reestruturação do gênero Deois Fennah; descrição de um novo gênero e de novas espécies (Homoptera, Cercopidae, Tomaspidinae)

Restructure of the genus Deois Fennah; description of a new genus and new species (Homoptera, Cercopidae, Tomaspidinae). The genus Deois Fennah is reviewed and some changes in the taxonomy are introduced. The genus and its four subgenera are redefined, having now the following composition: 1) subgenus Deois (Deois) with: D. (D.) correntina (Berg, 1879), D. (D.) grandis Sakakibara, 1979, D. (D.) knoblauchii (Berg, 1879) (formerly in D. (Pandysia)), D. (D.) morialis (China & Myers, 1934), D. (D.) mourei Cavichioli & Sakakibara, 1994, D. (D.) piraporae Sakakibara, 1979, D. (D.) pseudoflavopicta (Lallemand, 1938) comb. nov. (formerly in Mahanarva) = D. (D.) similis Sakakibara, 1979 syn. nov., D. (D.) rubropicta Sakakibara, 1979, D. (D.) spinulata sp. nov., D. (D.) terrea (Germar, 1821), D. (D.) uniformis (Distant, 1909). 2) subgenus Deois (Pandysia) with: D. (P.) bergi sp. nov., D. (P.) crenulata sp. nov., D. (P.) schach (Fabricius, 1787) = Sphenorhyna transiens Walker, 1851 syn. nov.. 3) Deois (Fennahia) with: D. (F.) coerulea (Lallemand, 1924), D. (F.) flexuosa (Walker, 1851). 4) Deois (Acanthodeois) with: D. (A.) flavopicta (Stål, 1854), Deois (A.) incompleta (Walker, 1851). The genus Orodamnis Fennah, 1953 stat. nov. (formerly Deois (Orodamnis)) with: Orodamnis rhynchosporae (China & Myers, 1934) comb. nov. The genus Deoisella gen. nov. is described for: Deoisella fasciata sp. nov. (type species) and Deoisella picklesi (China & Myers, 1934) comb. nov.

Records of Myodopsylla wolffsohni wolffsohni (Rothschild, 1903) (Siphonaptera, Ischnopsyllidae) on Myotis nigricans Schinz, 1821 (Chiroptera, Vespertilionidae), from the State of Paraná, Southern Brazil

The flea, Myodopsylla wolffsohni wolffsohni (Rothschild, 1903), had been recorded for the first time in the State of Paraná in 1940, on the bat, Myotis levis (I. Geoffroy, 1824). Previously, this species of flea had only been recorded on Myotis nigricans, in the Amazonian region. This is the second record of M. w. wolffsohni on M. nigricans in Brazil, and the first in the State of Paraná. Although this flea has been found on undetermined Chiroptera in the State of Santa Catarina, the present record represents the meridional limit of geographic distribution for the infestation on M. nigricans.