title: ÐÐ½Ð°Ð»Ð¸Ð· Ð°ÐºÑÐ¾Ð½Ð½Ð¾Ð³Ð¾ ÑÑÐ°Ð½ÑÐ¿Ð¾ÑÑÐ° Ð½Ð° Ð¼Ð¾Ð´ÐµÐ»Ð¸ Ð½ÐµÐ¹ÑÐ¾ÑÐ¿Ð°ÑÑÐ¸ÑÐµÑÐºÐ¾Ð¹ Ð¿Ð°ÑÐ°Ð»ÐµÐ³Ð¸Ð¸ (ÑÐ¾ÑÐ¼Ð° 39) Dr. melanogaster
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°
titleType: Subtitle
title: Analysis of axon transport on the spastic paralegia model (type SPG39) Dr. melanogaster
Creators: name: ÐÐ¼ÑÐ¹Ð´Ð¸Ð½Ð°, ÐÐ°ÑÑÑ
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: 2020
Subjects: swiss cheese
Ð°ÐºÑÐ¾Ð½Ð½ÑÐ¹ ÑÑÐ°Ð½ÑÐ¿Ð¾ÑÑ
ÑÐ¿Ð°ÑÑÐ¸ÑÐµÑÐºÐ°Ñ Ð¿Ð°ÑÐ°Ð¿Ð»ÐµÐ³Ð¸Ñ
neuropathy target esterase
Drosophila melanogaster
Ð¼Ð¸ÑÐ¾Ñ Ð¾Ð½Ð´ÑÐ¸Ñ
Ð½ÐµÐ¹ÑÐ¾Ð½
axonal transport
spastic paraplegia
mitochondria
neuron

description: ÐÐ´Ð½Ð¸Ð¼ Ð¸Ð· Ð¿ÐµÑÐ²ÑÑ Ð¾ÑÐºÑÑÑÑÑ Ð³ÐµÐ½Ð¾Ð² Drosophila melanogaster, Ð¼ÑÑÐ°ÑÐ¸Ð¸ Ð² ÐºÐ¾ÑÐ¾ÑÐ¾Ð¼ Ð¿ÑÐ¸Ð²Ð¾Ð´ÑÑ Ðº Ð½ÐµÐ¹ÑÐ¾Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð¸, ÑÐ²Ð»ÑÐµÑÑÑ Ð³ÐµÐ½ sws (swiss cheese). ÐÑÑÐ°ÑÐ¸Ð¸ Ð² Ð½ÐµÐ¼ ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·ÑÑÑÑÑ Ð¿ÑÐ¾Ð³ÑÐµÑÑÐ¸ÑÑÑÑÐµÐ¹ Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸ÐµÐ¹ Ð·ÑÐµÐ»Ð¾Ð¹ Ð½ÐµÑÐ²Ð½Ð¾Ð¹ ÑÐ¸ÑÑÐµÐ¼Ñ, Ð³Ð»Ð¸Ð°Ð»ÑÐ½ÑÐ¼ Ð³Ð¸Ð¿ÐµÑÐ¾Ð±ÑÑÑÑÐ²Ð°Ð½Ð¸ÐµÐ¼ Ð¸ Ð½ÐµÐ¹ÑÐ¾Ð½Ð°Ð»ÑÐ½ÑÐ¼ Ð°Ð¿Ð¾Ð¿ÑÐ¾Ð·Ð¾Ð¼. ÐÑÑÐ¾Ð»Ð¾Ð³Ð¾Ð¼ sws Ñ ÑÐµÐ»Ð¾Ð²ÐµÐºÐ° ÑÐ²Ð»ÑÐµÑÑÑ Ð³ÐµÐ½ NTE (Neuropathy Target Esterase). ÐÑÑÐ°ÑÐ¸Ð¸ Ð² ÐºÐ°ÑÐ°Ð»Ð¸ÑÐ¸ÑÐµÑÐºÐ¾Ð¼ Ð´Ð¾Ð¼ÐµÐ½Ðµ NTE Ð¿ÑÐ¸Ð²Ð¾Ð´ÑÑ Ðº ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð½Ð°ÑÐ»ÐµÐ´ÑÑÐ²ÐµÐ½Ð½Ð¾Ð¹ ÑÐ¿Ð°ÑÑÐ¸ÑÐµÑÐºÐ¾Ð¹ Ð¿Ð°ÑÐ°Ð¿Ð»ÐµÐ³Ð¸Ð¸ ÑÐ¸Ð¿Ð° 39. ÐÐ°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ðµ ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·ÑÐµÑÑÑ Ð¼ÐµÐ´Ð»ÐµÐ½Ð½Ð¾ ÑÐ°Ð·Ð²Ð¸Ð²Ð°ÑÑÐµÐ¹ÑÑ Ð´Ð¸ÑÑÐ°Ð»ÑÐ½Ð¾Ð¹ Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸ÐµÐ¹ Ð°ÐºÑÐ¾Ð½Ð¾Ð² Ð¼Ð¾ÑÐ¾ÑÐ½ÑÑ Ð½ÐµÐ¹ÑÐ¾Ð½Ð¾Ð², ÑÐ¾Ð¿ÑÑÑÑÐ²ÑÑÑÐµÐ¹ Ð°ÑÑÐ¾ÑÐ¸ÐµÐ¹ Ð¼ÑÑÑ Ð¸ Ð¿Ð°ÑÐ°Ð»Ð¸ÑÐ¾Ð¼ Ð²ÐµÑÑÐ½Ð¸Ñ Ð¸ Ð½Ð¸Ð¶Ð½Ð¸Ñ ÐºÐ¾Ð½ÐµÑÐ½Ð¾ÑÑÐµÐ¹. ÐÐ° ÑÐµÐ³Ð¾Ð´Ð½ÑÑÐ½Ð¸Ð¹ Ð´ÐµÐ½Ñ Ð¼ÐµÑÐ°Ð½Ð¸Ð·Ð¼Ñ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð´Ð°Ð½Ð½Ð¾Ð³Ð¾ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ñ Ð´Ð¾ ÐºÐ¾Ð½ÑÐ° Ð½Ðµ Ð¸Ð·ÑÑÐµÐ½Ñ. ÐÐ°Ð½Ð½Ð°Ñ ÑÐ°Ð±Ð¾ÑÐ° Ð¿Ð¾ÑÐ²ÑÑÐµÐ½Ð° Ð°Ð½Ð°Ð»Ð¸Ð·Ñ Ð°ÐºÑÐ¾Ð½Ð½Ð¾Ð³Ð¾ ÑÑÐ°Ð½ÑÐ¿Ð¾ÑÑÐ° Ð¼Ð¸ÑÐ¾ÑÐ¾Ð½Ð´ÑÐ¸Ð¹ Ð² Ð½ÐµÐ¹ÑÐ¾Ð½Ð°Ñ ÐºÑÑÐ»Ð° D. melanogaster Ñ Ð¾ÑÐ¾Ð±ÐµÐ¹ Ð»Ð¸Ð½Ð¸Ð¹ Ñ Ð³Ð¸Ð¿ÐµÑÑÐºÑÐ¿ÑÐµÑÑÐ¸ÐµÐ¹ Ð³ÐµÐ½Ð° NTE ÑÐµÐ»Ð¾Ð²ÐµÐºÐ° Ð½Ð° Ð¼Ð¾Ð´ÐµÐ»Ð¸ D. melanogaster, Ð° ÑÐ°ÐºÐ¶Ðµ ÐµÐ³Ð¾ Ð¾ÑÑÐ¾Ð»Ð¾Ð³Ð° - Ð³ÐµÐ½Ð° sws Drosophila (Ð¿Ð¾Ð´Ð°Ð²Ð»ÐµÐ½Ð½Ð°Ñ ÑÐºÑÐ¿ÑÐµÑÑÐ¸Ñ Ð¸ Ð³Ð¸Ð¿ÐµÑÑÐºÑÐ¿ÑÐµÑÑÐ¸Ñ). ÐÑÐ½Ð¾Ð²Ð½ÑÐ¼Ð¸ Ð·Ð°Ð´Ð°ÑÐ°Ð¼Ð¸ Ð¸ÑÑÐ»ÐµÐ´Ð¾Ð²Ð°Ð½Ð¸Ñ Ð±ÑÐ»Ð¸: Ð°Ð½Ð°Ð»Ð¸Ð· ÑÐ°ÑÐ¿ÑÐµÐ´ÐµÐ»ÐµÐ½Ð¸Ñ Ð¼Ð¸ÑÐ¾ÑÐ¾Ð½Ð´ÑÐ¸Ð¹ Ð¸ ÑÑÐ°Ð²Ð½ÐµÐ½Ð¸Ðµ ÑÑÐµÐ´Ð½Ð¸Ñ ÑÐºÐ¾ÑÐ¾ÑÑÐµÐ¹ Ð´Ð²Ð¸Ð¶ÐµÐ½Ð¸Ñ Ð¼Ð¸ÑÐ¾ÑÐ¾Ð½Ð´ÑÐ¸Ð¹ Ð² Ð½ÐµÐ¹ÑÐ¾Ð½Ð°Ñ ÐºÑÑÐ»Ð° Ð´ÑÐ¾Ð·Ð¾ÑÐ¸Ð»Ñ. Ð Ð°Ð±Ð¾ÑÐ° Ð±ÑÐ»Ð° Ð²ÑÐ¿Ð¾Ð»Ð½ÐµÐ½Ð° Ð½Ð° Ð±Ð°Ð·Ðµ Ð¤ÐÐÐ£ Â«ÐÐµÑÐµÑÐ±ÑÑÐ³ÑÐºÐ¸Ð¹ Ð¸Ð½ÑÑÐ¸ÑÑÑ ÑÐ´ÐµÑÐ½Ð¾Ð¹ ÑÐ¸Ð·Ð¸ÐºÐ¸ Ð¸Ð¼. Ð.Ð. ÐÐ¾Ð½ÑÑÐ°Ð½ÑÐ¸Ð½Ð¾Ð²Ð° ÐÐ°ÑÐ¸Ð¾Ð½Ð°Ð»ÑÐ½Ð¾Ð³Ð¾ Ð¸ÑÑÐ»ÐµÐ´Ð¾Ð²Ð°ÑÐµÐ»ÑÑÐºÐ¾Ð³Ð¾ ÑÐµÐ½ÑÑÐ° Â«ÐÑÑÑÐ°ÑÐ¾Ð²ÑÐºÐ¸Ð¹ Ð¸Ð½ÑÑÐ¸ÑÑÑÂ» Ð² Ð»Ð°Ð±Ð¾ÑÐ°ÑÐ¾ÑÐ¸Ð¸ ÑÐºÑÐ¿ÐµÑÐ¸Ð¼ÐµÐ½ÑÐ°Ð»ÑÐ½Ð¾Ð¹ Ð¸ Ð¿ÑÐ¸ÐºÐ»Ð°Ð´Ð½Ð¾Ð¹ Ð³ÐµÐ½ÐµÑÐ¸ÐºÐ¸ Ð¿Ð¾Ð´ ÑÑÐºÐ¾Ð²Ð¾Ð´ÑÑÐ²Ð¾Ð¼ Ð´Ð¾ÐºÑÐ¾ÑÐ° Ð±Ð¸Ð¾Ð»Ð¾Ð³Ð¸ÑÐµÑÐºÐ¸Ñ Ð½Ð°ÑÐº Ð¡Ð°ÑÐ°Ð½ÑÐµÐ²Ð¾Ð¹ Ð¡Ð²ÐµÑÐ»Ð°Ð½Ñ ÐÐ»Ð°Ð´Ð¸Ð¼Ð¸ÑÐ¾Ð²Ð½Ñ.
description: The Drosophila Swiss cheese (sws) mutant is characterized by progressive degeneration of the adult nervous system, glial hyperwrapping, and neuronal apoptosis. The sws homolog in humans is the NTE gene (Neuropathy Target Esterase). Mutations in the human NTE lead to the formation of one type of HSP (SPG39). The disease is characterized by slowly developing distal axonal degeneration of motor neurons and concomitant muscle atrophy and paralysis of the upper and lower extremities. Now mechanisms of these diseases are not completely understood. This work is devoted to the analysis of axon transport of mitochondria in wing neurons D. melanogaster in lines with overexpression of the human NTE gene on the D. melanogaster, as well as its homologue, the sws Drosophila gene (suppressed expression and overexpression). The main objectives of the study were to analyze the distribution of mitochondria and to compare the average velocities of mitochondria in neurons of the Drosophila wing. The work was fulfilled on the basis of Petersburg Nuclear Physics Institute Named by B.P. Konstantinov of National Research center "Kurchatov Institute" in the laboratory of experimental and applied genetics under the guidance of Doctor of Biological Sciences Svetlana Vladimirovna Sarantseva.