Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein.

Objective: The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV). Methods: Fifteen affected subjects with 129MM, 129MV, and 129VV underwent comparative evaluation at the National Prion Disease Pathology Surveillance Center for clinical, histopathologic, immunohistochemical, genotypical, and PrP characteristics. Results: Disease duration (between 22 and 45 months) was significantly different in the 129VV and 129MV subjects. Most other phenotypic features along with the PrP electrophoretic profile were similar but distinguishable in the 3 129 genotypes. A major difference laid in the sensitivity to protease digestion of the disease-associated PrP, which was high in 129VV but much lower, or altogether lacking, in 129MV and 129MM. This difference prompted the substitution of the original designation with ‘‘variably protease-sensitive prionopathy’’ (VPSPr). None of the subjects had mutations in the PrP gene coding region. Interpretation: Because all 3 129 genotypes are involved, and are associated with distinguishable phenotypes, VPSPr becomes the second sporadic prion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920. However, the characteristics of the abnormal prion protein suggest that VPSPr is different from typical prion diseases, and perhaps more akin to subtypes of Gerstmann-Straussler-Scheinker disease

Morfologia de sementes e desenvolvimento pós-seminal de Physalis angulata L Seed morphology and post-seminal development of Physalis angulata L

Physalis angulata é conhecida como camapu, mullaca ou juá-de-capote e pertence à família Solanaceae. Esta espécie possui importância medicinal, pela presença dos vitaesteróides conhecidos como fisalinas e, atualmente, na alimentação. Essa espécie se propaga facilmente por sementes, motivo pelo qual ela é tida como infestante de outras culturas. Nesse sentido, a identificação da espécie ainda nos estágios iniciais de crescimento confunde com as demais infestantes, tornando difícil a sua caracterização taxonômica. Assim, o objetivo deste trabalho foi caracterizar morfologicamente as sementes e o desenvolvimento pós-seminal de Physalis angulata. Os ensaios foram desenvolvidos no Laboratório de Germinação da Unidade Experimental Horto Florestal, Feira de Santana - BA. Para tanto foram realizadas análises morfométricas das sementes (4 repetições de 25) e analisadas suas características externas e internas com auxilio de microscópico estereoscópico. Para a descrição das plântulas da espécie foram utilizadas placas de petri contendo duas folhas de papel filtro (4 repetições de 20 sementes) e umedecidos com água destilada. As sementes foram colocadas em câmara de germinação à 35ºC (fotoperíodo de 12 horas) até a emissão dos cotilédones. A metodologia para descrição do desenvolvimento pós-seminal foi realizada em condições de casa de vegetação e consistiu em avaliações realizadas cinco dias após a semeadura (emissão dos cotilédones) e no intervalo de até 15 dias (emissão de eófilos e metáfilos) no qual foram utilizados 2 repetições de 20 vasos. As sementes apresentaram em média 1,55 mm de comprimento, 1,26 mm de largura e 0,43 mm de espessura. O período de análise foi suficiente para caracterizar as sementes e plântulas da espécie, permitindo a adoção de critérios de anormalidade.<br>Physalis angulata is also known as camapu, mullaca or juá-de-capote it belongs to the Solanaceae family. This species has its medicinal importance due to vitasteroids presence known as physalins, and currently due to its use as food. It propagates readily by seed, which is the reason why it is regarded as weeds in other crops. Accordingly, the identification of that species in the early stages of growth is indistinguishable from other weeds, hindering taxonomic characterization. Thus, the objective of this study was to morphologically characterize seeds and post-seminal development of Physalis angulata. The tests were developed in the Laboratory of Germination of the Experimental Unit/Nursery, in Feira de Santana/BA. Therefore, we have performed morphometric analysis of the seeds (4 replicates of 25 seeds) and we have analyzed their external and internal features with the aid of a stereoscopic microscope. For the description of the species seedlings it was used Petri dishes containing two sheets of filter paper (4 replicates of 20 seeds) and moistened with distilled water. The methodology for describing the post-seminal developmente was conduced under field conditions and consisted of evaluetions performed five days after sowing (inssuance of cotyledons) and the range of up to 15 days (and inssuing eophylls metaphylls) in which two replicates were used 20 pots. Assessments were performed five days after sowing (issuance of cotyledons) and ranging of up to 15 days (emission of eophylls and metaphylls). The seeds had an average of 1.55 mm long, 1.26 mm wide and 0.43 mm thick. The period of analysis was sufficient to characterize the seeds and seedlings of the species, applied as a criteria for abnormality