title: ÐÐ»Ð¸ÑÐ½Ð¸Ðµ Ð¼ÑÑÐ°ÑÐ¸Ð¸ H828Q CUA-1 Ð½Ð° ÑÑÐ²ÑÑÐ²Ð¸ÑÐµÐ»ÑÐ½Ð¾ÑÑÑ Ð½ÐµÐ¼Ð°ÑÐ¾Ð´ C. elegans Ðº Ð¸Ð¾Ð½Ð°Ð¼ Ð¼ÐµÐ´Ð¸
title: Ð²ÑÐ¿ÑÑÐºÐ½Ð°Ñ ÐºÐ²Ð°Ð»Ð¸ÑÐ¸ÐºÐ°ÑÐ¸Ð¾Ð½Ð½Ð°Ñ ÑÐ°Ð±Ð¾ÑÐ° Ð±Ð°ÐºÐ°Ð»Ð°Ð²ÑÐ°
titleType: Subtitle
title: Influence of the H828Q CUA-1 mutation on the sensitivity of C. elegans nematodes to copper ions treatment
Creators: name: ÐÐµÑÐ¾Ð²Ð°, ÐÐ»ÐµÐºÑÐ°Ð½Ð´Ñ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: 2020
Subjects: Ð¼ÐµÐ´Ñ
Ð³Ð¾Ð¼ÐµÐ¾ÑÑÐ°Ð· Ð¼ÐµÐ´Ð¸
Ð¼ÐµÐ´Ñ-ÑÑÐ°Ð½ÑÐ¿Ð¾ÑÑÐ¸ÑÑÑÑÐ°Ñ ÐÐ¢Ð¤-Ð°Ð·Ð° ÐÐ¢Ð 7Ð
Ð±Ð¾Ð»ÐµÐ·Ð½Ñ ÐÐ¸Ð»ÑÑÐ¾Ð½Ð°
Ð½ÐµÐ¼Ð°ÑÐ¾Ð´Ñ C. elegans
Ð±ÐµÐ»Ð¾Ðº CUA-1
copper
copper metabolism
copper homeostasis
copper-transporting ATPase ATP7B
Wilson's disease
C. elegans nematodes
CUA-1 protein

description: Ð¢ÐµÐ¼Ð° Ð²ÑÐ¿ÑÑÐºÐ½Ð¾Ð¹ ÐºÐ²Ð°Ð»Ð¸ÑÐ¸ÐºÐ°ÑÐ¸Ð¾Ð½Ð½Ð¾Ð¹ ÑÐ°Ð±Ð¾ÑÑ Â«ÐÐ»Ð¸ÑÐ½Ð¸Ðµ Ð¼ÑÑÐ°ÑÐ¸Ð¸ H828Q CUA-1 Ð½Ð° ÑÑÐ²ÑÑÐ²Ð¸ÑÐµÐ»ÑÐ½Ð¾ÑÑÑ Ð½ÐµÐ¼Ð°ÑÐ¾Ð´ C. elegans Ðº Ð¸Ð¾Ð½Ð°Ð¼ Ð¼ÐµÐ´Ð¸Â»
 ÐÐµÐ´Ñ (Cu) â Ð¶Ð¸Ð·Ð½ÐµÐ½Ð½Ð¾ Ð²Ð°Ð¶Ð½ÑÐ¹ Ð¼Ð¸ÐºÑÐ¾ÑÐ»ÐµÐ¼ÐµÐ½Ñ, ÑÐ²Ð»ÑÑÑÐ¸Ð¹ÑÑ ÑÑÑÑÐºÑÑÑÐ½ÑÐ¼ Ð¸ ÐºÐ°ÑÐ°Ð»Ð¸ÑÐ¸ÑÐµÑÐºÐ¸Ð¼ ÐºÐ¾ÑÐ°ÐºÑÐ¾ÑÐ¾Ð¼ Ð¼Ð½Ð¾Ð³Ð¸Ñ ÑÐµÑÐ¼ÐµÐ½ÑÐ¾Ð². ÐÑÐ¾Ð¼Ðµ ÑÐ¾Ð³Ð¾, Ð¸Ð¾Ð½Ñ Cu Ð½Ð°Ð¿ÑÑÐ¼ÑÑ ÑÑÐ°ÑÑÐ²ÑÑÑ Ð² ÑÐ¸Ð³Ð½Ð°Ð»Ð»Ð¸Ð½Ð³Ðµ. Ð ÑÐ¾ Ð¶Ðµ Ð²ÑÐµÐ¼Ñ ÑÐ²Ð¾Ð±Ð¾Ð´Ð½ÑÐµ Ð¸Ð¾Ð½Ñ Cu Ð¿ÑÐ¾Ð²Ð¾ÑÐ¸ÑÑÑÑ Ð¾ÐºÐ¸ÑÐ»Ð¸ÑÐµÐ»ÑÐ½ÑÐ¹ ÑÑÑÐµÑÑ. Ð¢ÑÐ°Ð½ÑÐ¿Ð¾ÑÑÐ½Ð°Ñ ÑÐ¸ÑÑÐµÐ¼Ð° Ð¼ÐµÐ´Ð¸ (Ð¢Ð¡Ð) ÑÐµÑÐ°ÐµÑ Ð·Ð°Ð´Ð°ÑÑ Ð¿Ð¾Ð´Ð´ÐµÑÐ¶Ð°Ð½Ð¸Ñ Ð±ÐµÐ·Ð¾Ð¿Ð°ÑÐ½Ð¾Ð³Ð¾ ÑÑÐ¾Ð²Ð½Ñ Cu Ð² Ð¾ÑÐ³Ð°Ð½Ð¸Ð·Ð¼Ðµ. ÐÐ¾Ð»ÐµÐ·Ð½Ñ ÐÐ¸Ð»ÑÑÐ¾Ð½Ð° (ÐÐ), ÐºÐ°Ðº Ð¸ ÑÑÐ´ Ð´ÑÑÐ³Ð¸Ñ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ð¹ ÑÐµÐ»Ð¾Ð²ÐµÐºÐ°, ÑÐ²ÑÐ·Ð°Ð½Ð° Ñ Ð½Ð°ÑÑÑÐµÐ½Ð¸ÑÐ¼Ð¸ Ð² Ð¢Ð¡Ð. ÐÐ ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·ÑÐµÑÑÑ Ð½Ð°ÐºÐ¾Ð¿Ð»ÐµÐ½Ð¸ÐµÐ¼ Cu Ð² Ð¿ÐµÑÐµÐ½Ð¸ Ð¸ Ð¼Ð¾Ð·Ð³Ðµ, Ð¿ÑÐ¸Ð²Ð¾Ð´ÑÑÐ¸Ð¼ Ðº ÑÐ¸ÑÑÐ¾Ð·Ñ Ð¸ Ð½ÐµÐ¹ÑÐ¾Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð¸. Ð ÐµÐ²ÑÐ¾Ð¿ÐµÐ¹ÑÐºÐ¾Ð¹ Ð¿Ð¾Ð¿ÑÐ»ÑÑÐ¸Ð¸ Ð½Ð°Ð¸Ð±Ð¾Ð»ÐµÐµ ÑÐ°ÑÑÐ¾Ð¹ Ð¿ÑÐ¸ÑÐ¸Ð½Ð¾Ð¹ ÐÐ ÑÐ²Ð»ÑÐµÑÑÑ Ð¼ÑÑÐ°ÑÐ¸Ñ H1069Q Ð² Ð±ÐµÐ»ÐºÐµ ÐÐ¢Ð 7Ð, Ð²ÑÑÑÐ°Ð¸Ð²Ð°ÑÑÐµÐ¼ Ð¸Ð¾Ð½Ñ Cu Ð² ÑÐµÑÑÐ»Ð¾Ð¿Ð»Ð°Ð·Ð¼Ð¸Ð½ Ð¸ ÑÐºÑÐºÑÐµÑÐ¸ÑÑÑÑÐµÐ¼ Ð¸Ð·Ð»Ð¸ÑÐºÐ¸ Cu Ð² Ð¶ÐµÐ»ÑÑ. Ð Ð´Ð°Ð½Ð½Ð¾Ð¹ ÑÐ°Ð±Ð¾ÑÐµ Ð² ÐºÐ°ÑÐµÑÑÐ²Ðµ Ð²Ð¾Ð·Ð¼Ð¾Ð¶Ð½Ð¾Ð¹ Ð¼Ð¾Ð´ÐµÐ»Ð¸ ÐÐ Ð¸Ð·ÑÑÐ°Ð»Ð¸ Ð½ÐµÐ¼Ð°ÑÐ¾Ð´Ñ C. elegans, Ñ Ð¼ÑÑÐ°ÑÐ¸ÐµÐ¹ H828Q Ð² Ð±ÐµÐ»ÐºÐµ CUA-1. ÐÐ·Ð²ÐµÑÑÐ½Ð¾, ÑÑÐ¾ Ð±ÐµÐ»Ð¾Ðº CUA-1 Ð³Ð¾Ð¼Ð¾Ð»Ð¾Ð³Ð¸ÑÐµÐ½ ATP7B, Ð° Ð¾ÑÑÐ°ÑÐ¾Ðº Ð828 Ð² Ð½ÑÐ¼ â ÑÑÐ½ÐºÑÐ¸Ð¾Ð½Ð°Ð»ÑÐ½ÑÐ¹ Ð°Ð½Ð°Ð»Ð¾Ð³ H1069. Ð¦ÐµÐ»Ñ ÑÐ°Ð±Ð¾ÑÑ - ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸ÑÑÐ¸ÐºÐ° ÑÐµÐ½Ð¾ÑÐ¸Ð¿Ð° C. elegans H828Q. Ð ÑÐ°Ð±Ð¾ÑÐµ Ð½ÐµÐ¼Ð°ÑÐ¾Ð´ ÐºÑÐ»ÑÑÐ¸Ð²Ð¸ÑÐ¾Ð²Ð°Ð»Ð¸ Ð² ÑÑÐµÐ´Ð°Ñ Ñ ÑÐ°Ð·Ð»Ð¸ÑÐ½ÑÐ¼Ð¸ ÐºÐ¾Ð½ÑÐµÐ½ÑÑÐ°ÑÐ¸ÑÐ¼Ð¸ Cu (0, 25, 50, 100 Ð¼ÐºÐ). ÐÐ¾ÐºÐ°Ð·Ð°Ð½Ð¾, ÑÑÐ¾ Ð¿ÑÐ¸ Ð±Ð°Ð·Ð¾Ð²ÑÑ ÐºÐ¾Ð½ÑÐµÐ½ÑÑÐ°ÑÐ¸ÑÑ Cu, ÑÐµÐ½Ð¾ÑÐ¸Ð¿ ÑÑÐ°Ð¼Ð¼Ð° H828Q Ð½Ðµ Ð¾ÑÐ»Ð¸ÑÐ°ÐµÑÑÑ Ð¾Ñ Ð´Ð¸ÐºÐ¾Ð³Ð¾. ÐÑÐ¸ Ð¿Ð¾Ð²ÑÑÐµÐ½Ð¸Ð¸ ÐºÐ¾Ð½ÑÐµÐ½ÑÑÐ°ÑÐ¸Ð¸ Cu Ð² ÑÑÐµÐ´Ðµ Ñ H828Q Ð·Ð°Ð¼ÐµÐ´Ð»ÑÐµÑÑÑ ÑÐºÐ¾ÑÐ¾ÑÑÑ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð»Ð¸ÑÐ¸Ð½Ð¾Ðº, ÑÐ½Ð¸Ð¶Ð°ÑÑÑÑ Ð¿ÑÐ¾Ð´Ð¾Ð»Ð¶Ð¸ÑÐµÐ»ÑÐ½Ð¾ÑÑÑ Ð¶Ð¸Ð·Ð½Ð¸, Ð¿Ð¾Ð´Ð²Ð¸Ð¶Ð½Ð¾ÑÑÑ Ð¸ Ð¾Ð±ÑÑÐ¼ ÑÐ¹ÑÐµÐºÐ»Ð°Ð´ÐºÐ¸. ÐÑÐ¸ Ð¼Ð½Ð¾Ð³Ð¸Ñ ÑÐ°ÑÑÐ¼Ð¾ÑÑÐµÐ½Ð½ÑÑ ÐºÐ¾Ð½ÑÐµÐ½ÑÑÐ°ÑÐ¸ÑÑ Cu, Ð¼ÑÑÐ°Ð½ÑÐ½ÑÐ¹ ÑÑÐ°Ð¼Ð¼ Ð½Ð°ÐºÐ°Ð¿Ð»Ð¸Ð²Ð°Ð» Cu Ð±Ð¾Ð»ÑÑÐµ, ÑÐµÐ¼ Ð´Ð¸ÐºÐ¸Ð¹. Ð¢Ð°ÐºÐ¸Ð¼ Ð¾Ð±ÑÐ°Ð·Ð¾Ð¼, Ð¸ÑÑÐ»ÐµÐ´Ð¾Ð²Ð°Ð½Ð½ÑÐµ Ð½ÐµÐ¼Ð°ÑÐ¾Ð´Ñ Ð´ÐµÐ¼Ð¾Ð½ÑÑÑÐ¸ÑÑÑÑ ÑÐµÐ½Ð¾ÑÐ¸Ð¿ ÐÐ, Ð²ÑÑÐ°Ð¶Ð°ÑÑÐ¸Ð¹ÑÑ Ð² Ð¿Ð¾Ð²ÑÑÐµÐ½Ð½Ð¾Ð¹ Ð°ÐºÐºÑÐ¼ÑÐ»ÑÑÐ¸Ð¸ Cu Ð¸ ÑÐ½Ð¸Ð¶ÐµÐ½Ð½ÑÑ ÑÐ¸Ð·Ð¸Ð¾Ð»Ð¾Ð³Ð¸ÑÐµÑÐºÐ¸Ñ Ð¿Ð°ÑÐ°Ð¼ÐµÑÑÐ°Ñ Ð² ÑÑÐ»Ð¾Ð²Ð¸ÑÑ ÐµÑ Ð¸Ð·Ð±ÑÑÐºÐ°.
description: The subject of the graduate qualification work is âInfluence of the H828Q CUA-1 mutation on the sensitivity of C. elegans nematodes to copper ions treatmentâ.
Copper (Cu) is a vital trace element that is a structural and catalytic cofactor of many enzymes. In addition, Cu is involved in a number of important metabolic processes. However, free Cu ions can induce an oxidative stress. The copper transport system (CTS) solves the problem of maintaining a safe level of Cu in the body. Wilson's disease (WD), as well as number of other human diseases, is associated with disorders in CTS. It is characterized by the accumulation of toxic Cu in the liver and brain, which leads to cirrhosis and neurodegeneration. In European population, WD is most often caused by a H1069Q mutation in the ATP7B protein, which inserts Cu in ceruloplasmin and excretes Cu excess ions into bile. In this work, C. elegans nematode with H828Q mutation in CUA-1 was studied as a possible model of WD. It is well known that CUA-1 protein is homologous to ATP7B, and the H828 residue in it, is a functional analogue of H1069. Characterization of the C. elegans H828Q phenotype was the aim of this work. Nematodes were cultured in media with different concentrations of Cu (0, 25, 50, 100 Î¼M/L). It was shown that at basal concentrations of Cu, the H828Q phenotype does not differ from the wild. While an increase of Cu concentration in the medium led to H828Q larvae development rate of, life span, mobility, and egg laying volume decrease. At most considered concentrations of Cu, the mutant strain accumulated more Cu than the wild. Thus, the studied nematodes demonstrate the WD phenotype, which is expressed in increased Cu accumulation and reduced physiological parameters under conditions of Cu excess.