First Report of Lysozyme Amyloidosis with p.F21L/T88N Amino Acid Substitutions in a Russian Family.

Lysozyme amyloidosis is caused by an amino acid substitution in the sequence of this protein. In our study, we described a clinical case of lysozyme amyloidosis in a Russian family. In our work, we described in detail the histological changes in tissues that appeared as a result of massive deposition of amyloid aggregates that affected almost all organ systems, with the exception of the central nervous system. We determined the type of amyloidosis and mutations using mass spectrometry. Using mass spectrometry, the protein composition of tissue samples of patient 1 (autopsy material) and patient 2 (biopsy material) with histologically confirmed amyloid deposits were analyzed. Amino acid substitutions p.F21L/T88N in the lysozyme sequence were identified in both sets of samples and confirmed by sequencing of the lysozyme gene of members of this family. We have shown the inheritance of these mutations in the lysozyme gene in members of the described family. For the first time, we discovered a mutation in the first exon p.F21L of the lysozyme gene, which, together with p.T88N amino acid substitution, led to amyloidosis in members of the studied family.

"Everything good against everything bad": traditional values in the search for new Russian national idea.

The purpose of the article is to explore origins, development and meaning of the Russian Orthodox Church's concept of traditional values in comparison with its interpreting and utilizing by the Russian political powers through the analysis of the main statements of the ROC's hierarchs, as well as of leading political figures in the period 2000s until nowadays. The following research questions are formulated: What are traditional values in the ROC's and state understanding, and what is the purpose of their promotion? What is the impact of traditional values discourse over people? The conclusion is made that traditional values are seen as one of substantial elements of the present-day Russian political and ideological conservatism and the core element of the identity construction. The ROC as the prominent and influential religious body is seen by political powers, as well as by general public, to be most notably linked to morality; and she seems to take for granted the status of moral authority. At the same time, there is a lack of reliable data, which make possible to estimate the real impact of traditional values over Russian people and the amount of those who share them.