title: Ð¡ÑÑÑÐºÑÑÑÐ½ÑÐµ Ñ Ð°ÑÐ°ÐºÑÐµÑÐ¸ÑÑÐ¸ÐºÐ¸ Ð±ÐµÐ»ÐºÐ¾Ð², Ð²Ð¾Ð²Ð»ÐµÑÑÐ½Ð½ÑÑ Ð² Ð½ÐµÐ¹ÑÐ¾Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð²Ð½ÑÐµ Ð¿ÑÐ¾ÑÐµÑÑÑ, Ð¾Ð¿ÑÐµÐ´ÐµÐ»ÑÑÑÐ¸Ðµ Ð¾ÑÐ¾Ð±ÐµÐ½Ð½Ð¾ÑÑÐ¸ Ð¸Ñ Ð¼ÐµÐ¶Ð¼Ð¾Ð»ÐµÐºÑÐ»ÑÑÐ½ÑÑ Ð²Ð·Ð°Ð¸Ð¼Ð¾Ð´ÐµÐ¹ÑÑÐ²Ð¸Ð¹
title: Ð½Ð°ÑÑÐ½ÑÐ¹ Ð´Ð¾ÐºÐ»Ð°Ð´
titleType: Subtitle
title: Structural characteristics of proteins involved in neurodegenerative diseases that determine their intermolecular interactions
Creators: name: ÐÐµÐ¼ÐºÐ¾Ð², ÐÐ»Ð°Ð´Ð¸Ð¼Ð¸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: 2018
Subjects: ÐÐµÐ»ÐºÐ¸
ÐÐµÐ¶Ð¼Ð¾Ð»ÐµÐºÑÐ»ÑÑÐ½Ð¾Ðµ Ð²Ð·Ð°Ð¸Ð¼Ð¾Ð´ÐµÐ¹ÑÑÐ²Ð¸Ðµ
Ð ÐµÐ½ÑÐ³ÐµÐ½Ð¾ÑÑÑÑÐºÑÑÑÐ½ÑÐ¹ Ð°Ð½Ð°Ð»Ð¸Ð·
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description: This work is focused on determination of the structural features of three proteins involved in neurodegenerative diseases: ataxin-3, huntingtin and sigma-1 receptor. Crystal structure of the C-terminal domain of ataxin-3 shows that polyglutamine tract can exist in two conformational states: random coil and alpha-helix. The polyQ helix is stabilized by two nets of hydrogen bonds. By synthesizing a library of peptoid compounds we identified a new peptoid HNP1 that selectively binds to huntingtin amino-terminal region (N17). It was shown that HNP1 suppresses aggregation of mutant huntingtin in vitro and is neuropreotective in cellular model of Huntingtonâs diseases. Sigma-1 receptor was reconstituted in model lipid âgiantâ liposomes. It was shown that S1R incorporation into bilayer leads to clustering on the receptor in cholesterol-containing liposomes. A putative cholesterol binding site was identified in the transmembrane region. Experiments shown that sterol-binding motif is required for proper targeting of the receptor to the mitochondria-associated membranes of the ER.