Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin ligh... more Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin light chains (LCs). Potential drivers of amyloid formation in this disease are post-translational modifications (PTMs) and the mutational changes that are inserted into the LCs by somatic hypermutation. Here we present the cryo electron microscopy (cryo-EM) structure of an ex vivo λ1-AL amyloid fibril whose deposits disrupt the ordered cardiomyocyte structure in the heart. The fibril protein contains six mutational changes compared to the germ line and three PTMs (disulfide bond, N-glycosylation and pyroglutamylation). Our data imply that the disulfide bond, glycosylation and mutational changes contribute to determining the fibril protein fold and help to generate a fibril morphology that is able to withstand proteolytic degradation inside the body.
Out of 28 wars fought in 1999, 19 were intra-state and antiregime wars; and out of these about 15... more Out of 28 wars fought in 1999, 19 were intra-state and antiregime wars; and out of these about 15 were or are being led with the aim of autonomy or secession. As we all know, the Caucasus is one of the hot spots of world policy with four wars waged, three of them frozen in for some time. All of them are wars for secession. Above these there are many more interethnic conflicts in Caucasia, which have erupted mainly on the question of the political-juridical status of certain territories. All of them have the potential to turn into violent clashes and even wars. This situation is all the more aggravated by the fact that in Georgia and Azerbaijan, the question of succession for the current presidencies remain in a state of uncertainty, which could lead to a period of political unstability in these countries as well as in the whole area.
... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständ... more ... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständig gesichert, die Aufsicht des Verteidi-gungsministers über das Militär gewährleistet sowie die Bedeutung der Militär-gerichte eingeschränkt. ...
Zeitschrift für Staats- und Europawissenschaften, 2005
... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständ... more ... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständig gesichert, die Aufsicht des Verteidi-gungsministers über das Militär gewährleistet sowie die Bedeutung der Militär-gerichte eingeschränkt. ...
... However, its development in Kist mountain villages occurred at the beginning of the 20th cent... more ... However, its development in Kist mountain villages occurred at the beginning of the 20th century only, when in 1909 Isa Efendi, a Naqshbandi preacher from Azerbaijan, travelled to Georgia to spread Naqshbandi mystical teaching and practice. ...
... However, its development in Kist mountain villages occurred at the beginning of the 20th cent... more ... However, its development in Kist mountain villages occurred at the beginning of the 20th century only, when in 1909 Isa Efendi, a Naqshbandi preacher from Azerbaijan, travelled to Georgia to spread Naqshbandi mystical teaching and practice. ...
Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin ligh... more Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin light chains (LCs). Potential drivers of amyloid formation in this disease are post-translational modifications (PTMs) and the mutational changes that are inserted into the LCs by somatic hypermutation. Here we present the cryo electron microscopy (cryo-EM) structure of an ex vivo λ1-AL amyloid fibril whose deposits disrupt the ordered cardiomyocyte structure in the heart. The fibril protein contains six mutational changes compared to the germ line and three PTMs (disulfide bond, N-glycosylation and pyroglutamylation). Our data imply that the disulfide bond, glycosylation and mutational changes contribute to determining the fibril protein fold and help to generate a fibril morphology that is able to withstand proteolytic degradation inside the body.
Out of 28 wars fought in 1999, 19 were intra-state and antiregime wars; and out of these about 15... more Out of 28 wars fought in 1999, 19 were intra-state and antiregime wars; and out of these about 15 were or are being led with the aim of autonomy or secession. As we all know, the Caucasus is one of the hot spots of world policy with four wars waged, three of them frozen in for some time. All of them are wars for secession. Above these there are many more interethnic conflicts in Caucasia, which have erupted mainly on the question of the political-juridical status of certain territories. All of them have the potential to turn into violent clashes and even wars. This situation is all the more aggravated by the fact that in Georgia and Azerbaijan, the question of succession for the current presidencies remain in a state of uncertainty, which could lead to a period of political unstability in these countries as well as in the whole area.
... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständ... more ... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständig gesichert, die Aufsicht des Verteidi-gungsministers über das Militär gewährleistet sowie die Bedeutung der Militär-gerichte eingeschränkt. ...
Zeitschrift für Staats- und Europawissenschaften, 2005
... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständ... more ... Als Mehmet II. ... Des Weiteren ist die Budgetkon-trolle durch das Parlament nahezu vollständig gesichert, die Aufsicht des Verteidi-gungsministers über das Militär gewährleistet sowie die Bedeutung der Militär-gerichte eingeschränkt. ...
... However, its development in Kist mountain villages occurred at the beginning of the 20th cent... more ... However, its development in Kist mountain villages occurred at the beginning of the 20th century only, when in 1909 Isa Efendi, a Naqshbandi preacher from Azerbaijan, travelled to Georgia to spread Naqshbandi mystical teaching and practice. ...
... However, its development in Kist mountain villages occurred at the beginning of the 20th cent... more ... However, its development in Kist mountain villages occurred at the beginning of the 20th century only, when in 1909 Isa Efendi, a Naqshbandi preacher from Azerbaijan, travelled to Georgia to spread Naqshbandi mystical teaching and practice. ...
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Papers by Raoul Motika