title: ÐÐ»Ð¸ÑÐ½Ð¸Ðµ Ð´Ð¸ÑÑÑÐ½ÐºÑÐ¸Ð¸ Ð³Ð»ÑÐºÐ¾ÑÐµÑÐµÐ±ÑÐ¾Ð·Ð¸Ð´Ð°Ð·Ñ Ð½Ð° Ð¿ÑÐ» ÑÐºÑÑÑÐ°ÐºÐ»ÐµÑÐ¾ÑÐ½ÑÑ Ð²ÐµÐ·Ð¸ÐºÑÐ» Ð¿Ð»Ð°Ð·Ð¼Ñ ÐºÑÐ¾Ð²Ð¸ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ Ð±Ð¾Ð»ÐµÐ·Ð½ÑÑ ÐÐ¾ÑÐµ
title: Ð²ÑÐ¿ÑÑÐºÐ½Ð°Ñ ÐºÐ²Ð°Ð»Ð¸ÑÐ¸ÐºÐ°ÑÐ¸Ð¾Ð½Ð½Ð°Ñ ÑÐ°Ð±Ð¾ÑÐ° Ð¼Ð°Ð³Ð¸ÑÑÑÐ° Ð¿Ð¾ Ð½Ð°Ð¿ÑÐ°Ð²Ð»ÐµÐ½Ð¸Ñ 03.04.02 - Ð¤Ð¸Ð·Ð¸ÐºÐ° ; 03.04.02_02 - ÐÐ¸Ð¾ÑÐ¸Ð·Ð¸ÐºÐ°
titleType: Subtitle
Creators: name: ÐÐ°ÑÐ°ÐµÐ²Ð°, ÐÑÐ¸Ð·Ð°
Publisher: name: Ð¡Ð°Ð½ÐºÑ-ÐÐµÑÐµÑÐ±ÑÑÐ³ÑÐºÐ¸Ð¹ Ð¿Ð¾Ð»Ð¸ÑÐµÑÐ½Ð¸ÑÐµÑÐºÐ¸Ð¹ ÑÐ½Ð¸Ð²ÐµÑÑÐ¸ÑÐµÑ ÐÐµÑÑÐ° ÐÐµÐ»Ð¸ÐºÐ¾Ð³Ð¾
Year: 2019
Subjects: Ð»Ð¸Ð·Ð¾ÑÐ¾Ð¼Ð½ÑÐµ Ð±Ð¾Ð»ÐµÐ·Ð½Ð¸ Ð½Ð°ÐºÐ¾Ð¿Ð»ÐµÐ½Ð¸Ñ
Ð±Ð¾Ð»ÐµÐ·Ð½Ñ Ð³Ð¾ÑÐµ
Ð³Ð»ÑÐºÐ¾ÑÐµÑÐµÐ±ÑÐ¾Ð·Ð¸Ð´Ð°Ð·Ð°
GBA1
ÐºÐ»ÐµÑÐºÐ¸ Ð³Ð¾ÑÐµ
ÑÐºÑÑÑÐ°ÐºÐ»ÐµÑÐ¾ÑÐ½ÑÐµ Ð²ÐµÐ·Ð¸ÐºÑÐ»Ñ
ÑÐºÐ·Ð¾ÑÐ¾Ð¼Ñ
Ð¿Ð»Ð°Ð·Ð¼Ð° ÐºÑÐ¾Ð²Ð¸
Ð½Ð°ÑÐ»ÐµÐ´ÑÑÐ²ÐµÐ½Ð½ÑÐµ Ð±Ð¾Ð»ÐµÐ·Ð½Ð¸
Ð¼ÑÑÐ°ÑÐ¸Ð¸

description: ÐÐ¾Ð»ÐµÐ·Ð½Ñ ÐÐ¾ÑÐµ (ÐÐ) â Ð½Ð°Ð¸Ð±Ð¾Ð»ÐµÐµ ÑÐ°ÑÐ¿ÑÐ¾ÑÑÑÐ°Ð½ÐµÐ½Ð½Ð°Ñ Ð»Ð¸Ð·Ð¾ÑÐ¾Ð¼Ð½Ð°Ñ Ð±Ð¾Ð»ÐµÐ·Ð½Ñ Ð½Ð°ÐºÐ¾Ð¿Ð»ÐµÐ½Ð¸Ñ, ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·ÑÑÑÐ°ÑÑÑ ÑÐ½Ð¸Ð¶ÐµÐ½Ð¸ÐµÐ¼ Ð°ÐºÑÐ¸Ð²Ð½Ð¾ÑÑÐ¸ Ð»Ð¸Ð·Ð¾ÑÐ¾Ð¼Ð°Ð»ÑÐ½Ð¾Ð³Ð¾ ÑÐµÑÐ¼ÐµÐ½ÑÐ° Ð³Ð»ÑÐºÐ¾ÑÐµÑÐµÐ±ÑÐ¾Ð·Ð¸Ð´Ð°Ð·Ñ (GCase), ÑÑÐ¾ Ð¾Ð±ÑÑÐ»Ð¾Ð²Ð»ÐµÐ½Ð¾ Ð¼ÑÑÐ°ÑÐ¸ÑÐ¼Ð¸ Ð² ÐºÐ¾Ð´Ð¸ÑÑÑÑÐµÐ¼ Ð´Ð°Ð½Ð½ÑÐ¹ ÑÐµÑÐ¼ÐµÐ½Ñ Ð³ÐµÐ½Ðµ GBA1. Ð¤ÑÐ½ÐºÑÐ¸Ð¾Ð½Ð¸ÑÐ¾Ð²Ð°Ð½Ð¸Ðµ Ð»Ð¸Ð·Ð¾ÑÐ¾Ð¼ Ð²Ð·Ð°Ð¸Ð¼Ð¾ÑÐ²ÑÐ·Ð°Ð½Ð¾ Ñ ÑÐµÐ³ÑÐ»ÑÑÐ¸ÐµÐ¹ ÑÐµÐºÑÐµÑÐ¸Ð¸ ÑÐºÑÑÑÐ°ÐºÐ»ÐµÑÐ¾ÑÐ½ÑÑ Ð²ÐµÐ·Ð¸ÐºÑÐ» (Ð­Ð). ÐÐ° ÑÐµÐ³Ð¾Ð´Ð½ÑÑÐ½Ð¸Ð¹ Ð´ÐµÐ½Ñ, Ð¾ÑÑÐ°ÐµÑÑÑ Ð½ÐµÐ¸Ð·Ð²ÐµÑÑÐ½ÑÐ¼, Ð²Ð»Ð¸ÑÐµÑ Ð»Ð¸ Ð´Ð¸ÑÑÑÐ½ÐºÑÐ¸Ñ Ð»Ð¸Ð·Ð¾ÑÐ¾Ð¼, Ð²Ð¾Ð·Ð½Ð¸ÐºÐ°ÑÑÐ°Ñ Ð¿ÑÐ¸ ÐÐ, Ð½Ð° Ð¿ÑÐ» Ð­Ð Ð¿Ð»Ð°Ð·Ð¼Ñ ÐºÑÐ¾Ð²Ð¸. Ð Ð´Ð°Ð½Ð½Ð¾Ð¹ ÑÐ°Ð±Ð¾ÑÐµ Ð±ÑÐ»Ð¸ Ð¾ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·Ð¾Ð²Ð°Ð½Ñ Ð­Ð, Ð¿Ð¾Ð»ÑÑÐµÐ½Ð½ÑÐµ Ð¸Ð· Ð¿Ð»Ð°Ð·Ð¼Ñ ÐºÑÐ¾Ð²Ð¸ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ ÐÐ Ð¸ Ð»Ð¸Ñ ÐºÐ¾Ð½ÑÑÐ¾Ð»ÑÐ½Ð¾Ð¹ Ð³ÑÑÐ¿Ð¿Ñ, Ð¼ÐµÑÐ¾Ð´Ð°Ð¼Ð¸ ÐºÑÐ¸Ð¾-ÑÐ»ÐµÐºÑÑÐ¾Ð½Ð½Ð¾Ð¹ Ð¼Ð¸ÐºÑÐ¾ÑÐºÐ¾Ð¿Ð¸Ð¸, Ð°Ð½Ð°Ð»Ð¸Ð·Ð° ÑÑÐ°ÐµÐºÑÐ¾ÑÐ¸Ð¸ Ð½Ð°Ð½Ð¾ÑÐ°ÑÑÐ¸Ñ (NTA), Ð´Ð¸Ð½Ð°Ð¼Ð¸ÑÐµÑÐºÐ¾Ð³Ð¾ ÑÐ²ÐµÑÐ¾ÑÐ°ÑÑÐµÑÐ½Ð¸Ñ (ÐÐ¡Ð ) Ð¸ dot-blot Ð¼ÐµÑÐ¾Ð´Ð¾Ð¼. Ð¢Ð°ÐºÐ¶Ðµ Ð±ÑÐ»Ð° Ð¿ÑÐ¾Ð¸Ð·Ð²ÐµÐ´ÐµÐ½Ð° ÐºÐ¾Ð»Ð¸ÑÐµÑÑÐ²ÐµÐ½Ð½Ð°Ñ Ð¾ÑÐµÐ½ÐºÐ° ÑÐºÐ·Ð¾ÑÐ¾Ð¼ Ð² Ð¿Ð»Ð°Ð·Ð¼Ðµ ÐºÑÐ¾Ð²Ð¸ Ð¿ÑÐµÐ´ÑÑÐ°Ð²Ð¸ÑÐµÐ»ÐµÐ¹ Ð¸ÑÑÐ»ÐµÐ´ÑÐµÐ¼ÑÑ Ð³ÑÑÐ¿Ð¿ Ð¼ÐµÑÐ¾Ð´Ð¾Ð¼ Ð¿ÑÐ¾ÑÐ¾ÑÐ½Ð¾Ð¹ ÑÐ¸ÑÐ¾Ð¼ÐµÑÑÐ¸Ð¸. ÐÑÐ»Ð¾ Ð¿ÑÐ¾Ð´ÐµÐ¼Ð¾Ð½ÑÑÑÐ¸ÑÐ¾Ð²Ð°Ð½Ð¾ ÑÐ°Ð·Ð½Ð¾Ð¾Ð±ÑÐ°Ð·Ð¸Ðµ Ð¼Ð¾ÑÑÐ¾Ð»Ð¾Ð³Ð¸Ð¸ Ð­Ð Ð¿Ð»Ð°Ð·Ð¼Ñ ÐºÑÐ¾Ð²Ð¸. ÐÐ¾ÐºÐ°Ð·Ð°Ð½Ð¾ ÑÐ²ÐµÐ»Ð¸ÑÐµÐ½Ð¸Ðµ ÑÐ¸ÑÐ»Ð° Ð­Ð, Ð¸Ð¼ÐµÑÑÐ¸Ñ Ð´Ð¸Ð°Ð¼ÐµÑÑ 40 Ð½Ð¼ â 120 Ð½Ð¼, Ð² Ð³ÑÑÐ¿Ð¿Ðµ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ ÐÐ Ð¿Ð¾ ÑÑÐ°Ð²Ð½ÐµÐ½Ð¸Ñ Ñ Ð³ÑÑÐ¿Ð¿Ð¾Ð¹ ÐºÐ¾Ð½ÑÑÐ¾Ð»Ñ. Ð¢Ð°ÐºÐ¶Ðµ Ð¾Ð±Ð½Ð°ÑÑÐ¶ÐµÐ½Ð¾ ÑÐ²ÐµÐ»Ð¸ÑÐµÐ½Ð¸Ðµ ÑÑÐ¾Ð²Ð½Ñ Ð¿Ð¾Ð²ÐµÑÑÐ½Ð¾ÑÑÐ½Ð¾Ð³Ð¾ Ð¼Ð°ÑÐºÐµÑÐ° ÑÐºÐ·Ð¾ÑÐ¾Ð¼ CD9 Ð² Ð³ÑÑÐ¿Ð¿Ðµ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ ÐÐ Ð¿Ð¾ ÑÑÐ°Ð²Ð½ÐµÐ½Ð¸Ñ Ñ ÐºÐ¾Ð½ÑÑÐ¾Ð»ÐµÐ¼. Ð¢Ð°ÐºÐ¸Ð¼ Ð¾Ð±ÑÐ°Ð·Ð¾Ð¼, Ð¼Ð¾Ð¶Ð½Ð¾ Ð¿ÑÐµÐ´Ð¿Ð¾Ð»Ð¾Ð¶Ð¸ÑÑ Ð¾ Ð²Ð»Ð¸ÑÐ½Ð¸Ñ Ð´Ð¸ÑÑÑÐ½ÐºÑÐ¸Ð¸ GBA Ð½Ð° ÑÑÐ¾Ð²ÐµÐ½Ñ ÑÐºÐ·Ð¾ÑÐ¾Ð¼ Ð² Ð¿Ð»Ð°Ð·Ð¼Ðµ ÐºÑÐ¾Ð²Ð¸ Ð¿ÑÐ¸ ÐÐ.
description: Gaucher disease (GD) â is the most common form of lysosomal storage diseases caused by mutations in the glucocerebrosidase (GBA) gene. Lysosome functionality is critical for the regulation of extracellular vesicules (EVs) secretion and content. However, it is unknown whether lysosomal dysfunction found in GD patients influence plasma pull of EVs and their protein composition. In this work EVs purified from blood plasma of GD patients and controls were characterized using cryo-electron microscopy, nanoparticle tracking and dynamic light scattering. Quantitative analysis of CD9 exosomal marker on the surface of the isolated EVs was carried out by flow cytometry. No difference was found between GD and controls in particle size and concentration evaluated by NTA. However, DLS showed increased proportion of exosomes (40-120nm) in EVs fraction from GD patients compared to controls (p&lt;0.001). We also showed an increased EVs expressing exosomal marker CD9 in GD compared to controls. 
Conclusions: Lysosomal dysfunction in GD patients might lead to an increase in level of plasma exosomes.