Additions to the lichen biota of Berezovye Islands, Leningrad Region, Russia

66 species of lichens, 9 lichenicolous fungi and 4 non-lichenized saprobic fungi are reported as new to the Berezovye Islands (Leningrad Region, Russia). The updated lichen biota of the archipelago comprises 356 species. Caloplaca maritima, Lecanora compallens, Verrucaria boblensis and Xanthoriicola physciae are reported for the first time for Russia, Lecanora aitema – for European Russia, Lecanora semipallida and Lichenodiplis lecanorae – for North-Western European Russia. Absconditella sphagnorum, Chaenothecopsis vainioana, Lempholemma polyanthes and Opegrapha niveoatra are new to the Leningrad Region, Arthonia byssacea and Lecanora subrugosa – to the Western Leningrad Region.

Genesis of Mammalian Prions: From Non-infectious Amyloid Fibrils to a Transmissible Prion Disease

The transmissible agent of prion disease consists of a prion protein in its abnormal, β-sheet rich state (PrPSc), which is capable of replicating itself according to the template-assisted mechanism. This mechanism postulates that the folding pattern of a newly recruited polypeptide chain accurately reproduces that of a PrPSc template. Here we report that authentic PrPSc and transmissible prion disease can be generated de novo in wild type animals by recombinant PrP (rPrP) amyloid fibrils, which are structurally different from PrPSc and lack any detectable PrPSc particles. When induced by rPrP fibrils, a long silent stage that involved two serial passages preceded development of the clinical disease. Once emerged, the prion disease was characterized by unique clinical, neuropathological, and biochemical features. The long silent stage to the disease was accompanied by significant transformation in neuropathological properties and biochemical features of the proteinase K-resistant PrP material (PrPres) before authentic PrPSc evolved. The current work illustrates that transmissible prion diseases can be induced by PrP structures different from that of authentic PrPSc and suggests that a new mechanism different from the classical templating exists. This new mechanism designated as “deformed templating” postulates that a change in the PrP folding pattern from the one present in rPrP fibrils to an alternative specific for PrPSc can occur. The current work provides important new insight into the mechanisms underlying genesis of the transmissible protein states and has numerous implications for understanding the etiology of neurodegenerative diseases

The Russian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Russian language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 100 JIA patients (25% systemic, 19% oligoarticular, 38% RF-negative polyarthritis, 18% other categories) and 198 healthy children, were enrolled in two centres. The JAMAR components discriminated healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Russian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research

Russian Sentence Corpus: Benchmark Measures of Eye Movements in Reading in Russian

This article introduces a new corpus of eye movements in silent reading—the Russian Sentence Corpus (RSC). Russian uses the Cyrillic script, which has not yet been investigated in cross-linguistic eye movement research. As in every language studied so far, we confirmed the expected effects of low-level parameters, such as word length, frequency, and predictability, on the eye movements of skilled Russian readers. These findings allow us to add Slavic languages using Cyrillic script (exemplified by Russian) to the growing number of languages with different orthographies, ranging from the Roman-based European languages to logographic Asian ones, whose basic eye movement benchmarks conform to the universal comparative science of reading (Share, 2008). We additionally report basic descriptive corpus statistics and three exploratory investigations of the effects of Russian morphology on the basic eye movement measures, which illustrate the kinds of questions that researchers can answer using the RSC. The annotated corpus is freely available from its project page at the Open Science Framework: https://osf.io/x5q2r/

Social and Labor Adaptation of People with Disabilities by Means of Production of Items of Folk Art: Evidence from Sociological Study

The article looks at the issues of integration of people with disabilities into a contemporary Russian society by means of utilization of their labor potential in the production of items of folk art with consideration of experience of this kind of activities in Vietnam. The relevance of this theme arises due to the lack of a much needed attention on the part of the government in formation of socially adequate and non-discriminatory conditions for life and activities of people with disabilities, which would allow them to overcome an image of a passive and dependent disability in the mass consciousness and disabled peoples' own self-consciousness. The most important condition of a successful integration of people with disabilities is their professional self-determination and employment. The study shows that people with disabilities face significant difficulties in identification of their place in life from the perspective of their professional employment. A priority area of employment of people with disabilities is their involvement in decorative arts, which encourages their full integration in a society, as most often they cannot adapt to the usual conditions of life and cannot compete on the labor market as equals with people without disabilities. Considering the fact that labor rehabilitation of people with disabilities by means of their involvement with decorative arts is not sufficiently spread in Russia there is a need to actively spread the media message about the advances of the people with disabilities in Vietnam in the sphere of production of items in decorative arts. Keywords: People with disabilities, social integration, labor rehabilitation, social policy, labor market, professional niches, folk art JEL Classifications: I14; J71; J2

Excretion of Transmissible Spongiform Encephalopathy Infectivity in Urine

Infectivity in hamster urine indicates a possible route of horizontal transmission of natural sheep scrapie and poses a potential risk in human urine-derived pharmaceuticals

Highly Efficient Protein Misfolding Cyclic Amplification

Protein misfolding cyclic amplification (PMCA) provides faithful replication of mammalian prions in vitro and has numerous applications in prion research. However, the low efficiency of conversion of PrPC into PrPSc in PMCA limits the applicability of PMCA for many uses including structural studies of infectious prions. It also implies that only a small sub-fraction of PrPC may be available for conversion. Here we show that the yield, rate, and robustness of prion conversion and the sensitivity of prion detection are significantly improved by a simple modification of the PMCA format. Conducting PMCA reactions in the presence of Teflon beads (PMCAb) increased the conversion of PrPC into PrPSc from ∼10% to up to 100%. In PMCAb, a single 24-hour round consistently amplified PrPSc by 600-700-fold. Furthermore, the sensitivity of prion detection in one round (24 hours) increased by 2-3 orders of magnitude. Using serial PMCAb, a 1012-fold dilution of scrapie brain material could be amplified to the level detectible by Western blotting in 3 rounds (72 hours). The improvements in amplification efficiency were observed for the commonly used hamster 263K strain and for the synthetic strain SSLOW that otherwise amplifies poorly in PMCA. The increase in the amplification efficiency did not come at the expense of prion replication specificity. The current study demonstrates that poor conversion efficiencies observed previously have not been due to the scarcity of a sub-fraction of PrPC susceptible to conversion nor due to limited concentrations of essential cellular cofactors required for conversion. The new PMCAb format offers immediate practical benefits and opens new avenues for developing fast ultrasensitive assays and for producing abundant quantities of PrPSc in vitro

Recombinant prion protein induces a new transmissible prion disease in wild-type animals

Prion disease is a neurodegenerative malady, which is believed to be transmitted via a prion protein in its abnormal conformation (PrPSc). Previous studies have failed to demonstrate that prion disease could be induced in wild-type animals using recombinant prion protein (rPrP) produced in Escherichia coli. Here, we report that prion infectivity was generated in Syrian hamsters after inoculating full-length rPrP that had been converted into the cross-β-sheet amyloid form and subjected to annealing. Serial transmission gave rise to a disease phenotype with highly unique clinical and neuropathological features. Among them were the deposition of large PrPSc plaques in subpial and subependymal areas in brain and spinal cord, very minor lesioning of the hippocampus and cerebellum, and a very slow progression of disease after onset of clinical signs despite the accumulation of large amounts of PrPSc in the brain. The length of the clinical duration is more typical of human and large animal prion diseases, than those of rodents. Our studies establish that transmissible prion disease can be induced in wild-type animals by inoculation of rPrP and introduce a valuable new model of prion diseases