title: ÐÐ°ÑÑÑÐµÐ½Ð¸Ñ ÑÐ¸Ð½Ð°Ð¿ÑÐ¸ÑÐµÑÐºÐ¾Ð¹ Ð°ÐºÑÐ¸Ð²Ð°ÑÐ¸Ð¸ ÐºÐ»ÐµÑÐ¾Ðº ÐÑÑÐºÐ¸Ð½ÑÐµ Ð¼Ð¾Ð·Ð¶ÐµÑÐºÐ° ÑÐ¸ÑÑÐµÐ¼Ð¾Ð¹ Ð°ÑÑÐµÑÐµÐ½ÑÐ½ÑÑ Ð²Ð¾Ð»Ð¾ÐºÐ¾Ð½ Ð² Ð¿Ð°ÑÐ¾Ð³ÐµÐ½ÐµÐ·Ðµ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ð¹ Ð¿Ð¾Ð»Ð¸Ð³Ð»ÑÑÐ°Ð¼Ð¸Ð½Ð¾Ð²Ð¾Ð³Ð¾ ÑÑÐ°ÐºÑÐ°
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: Disruptions of cerebellar Purkinje cells synaptic activation by the system of afferent fibers in the pathogenesis of polyglutamine disorders
Creators: name: ÐÐ°Ð²ÑÐ¸Ð»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: Ð¼Ð¾Ð·Ð¶ÐµÑÐ¾Ðº
ÐºÐ»ÐµÑÐºÐ¸ ÐÑÑÐºÐ¸Ð½ÑÐµ
ÑÐ¿Ð¸Ð½Ð¾ÑÐµÑÐµÐ±ÐµÐ»Ð»ÑÑÐ½ÑÐµ Ð°ÑÐ°ÐºÑÐ¸Ð¸
Ð±Ð¾Ð»ÐµÐ·Ð½Ñ Ð¥Ð°Ð½ÑÐ¸Ð½Ð³ÑÐ¾Ð½Ð°
ÑÑÐ°Ð½ÑÐ³ÐµÐ½Ð½ÑÐµ Ð¶Ð¸Ð²Ð¾ÑÐ½ÑÐµ
ÐºÐ°Ð»Ð¸ÐµÐ²ÑÐµ ÐºÐ°Ð½Ð°Ð»Ñ
Ñ Ð»Ð¾ÑÐ·Ð¾ÐºÑÐ°Ð·Ð¾Ð½
cerebellum
Purkinje cells
spinocerebellar ataxias
Huntingtonâs disease
transgenic animals
potassium channels
chlorzoxazone

description: ÐÐ¾Ð»ÐµÐ·Ð½Ñ Ð¥Ð°Ð½ÑÐ¸Ð½Ð³ÑÐ¾Ð½Ð° (ÐÐ¥) Ð¸ Ð½ÐµÐºÐ¾ÑÐ¾ÑÑÐµ Ð²Ð¸Ð´Ñ Ð°ÑÑÐ¾ÑÐ¾Ð¼Ð½Ð¾-Ð´Ð¾Ð¼Ð¸Ð½Ð°Ð½ÑÐ½ÑÑ ÑÐ¿Ð¸Ð½Ð¾ÑÐµÑÐµÐ±ÐµÐ»Ð»ÑÑÐ½ÑÑ Ð°ÑÐ°ÐºÑÐ¸Ð¹ (Ð¡Ð¦Ð) ÑÐ²Ð»ÑÑÑÑÑ ÑÐ°Ð¼ÑÐ¼Ð¸ Ð¸Ð·Ð²ÐµÑÑÐ½ÑÐ¼Ð¸ ÑÑÐµÐ´Ð¸ Ð½ÐµÐ¹ÑÐ¾Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð²Ð½ÑÑ Ð¿Ð¾Ð»Ð¸Ð³Ð»ÑÑÐ°Ð¼Ð¸Ð½Ð¾Ð²ÑÑ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ð¹. ÐÐ°Ðº Ð¿ÑÐ¸ ÐÐ¥, ÑÐ°Ðº Ð¸ Ð¿ÑÐ¸ Ð¡Ð¦Ð2 Ð½Ð°Ð±Ð»ÑÐ´Ð°ÐµÑÑÑ Ð¿Ð¾ÑÐ°Ð¶ÐµÐ½Ð¸Ðµ ÐºÐ»ÐµÑÐ¾Ðº ÐÑÑÐºÐ¸Ð½ÑÐµ (ÐÐ) ÐºÐ¾ÑÑ Ð¼Ð¾Ð·Ð¶ÐµÑÐºÐ° Ð¸ Ð½Ð°ÑÑÑÐµÐ½Ð¸Ñ Ð¼Ð¾ÑÐ¾ÑÐ½ÑÑ ÑÑÐ½ÐºÑÐ¸Ð¹. ÐÐ·Ð²ÐµÑÑÐ½Ð¾, ÑÑÐ¾ ÐºÐ°Ð»ÑÑÐ¸Ð¹-Ð°ÐºÑÐ¸Ð²Ð¸ÑÑÐµÐ¼ÑÐµ ÐºÐ°Ð»Ð¸ÐµÐ²ÑÐµ ÐºÐ°Ð½Ð°Ð»Ñ Ð¼Ð°Ð»Ð¾Ð¹ Ð¿ÑÐ¾Ð²Ð¾Ð´Ð¸Ð¼Ð¾ÑÑÐ¸ (SK ÐºÐ°Ð½Ð°Ð»Ñ) Ð¾ÑÑÑÐµÑÑÐ²Ð»ÑÑÑ ÐºÐ¾Ð½ÑÑÐ¾Ð»Ñ Ð¿ÐµÐ¹ÑÐ¼ÐµÐ¹ÐºÐµÑÐ½Ð¾Ð¹ Ð°ÐºÑÐ¸Ð²Ð½Ð¾ÑÑÐ¸ ÐÐ. ÐÑÐ¿Ð¾Ð»ÑÐ·ÑÑ Ð¼ÐµÑÐ¾Ð´ Ð²Ð½ÐµÐºÐ»ÐµÑÐ¾ÑÐ½Ð¾Ð¹ ÑÐµÐ³Ð¸ÑÑÑÐ°ÑÐ¸Ð¸, Ð¼Ñ Ð¿ÑÐ¾Ð°Ð½Ð°Ð»Ð¸Ð·Ð¸ÑÐ¾Ð²Ð°Ð»Ð¸ ÑÐ»ÐµÐºÑÑÐ¾ÑÐ¸Ð·Ð¸Ð¾Ð»Ð¾Ð³Ð¸ÑÐµÑÐºÐ¸Ðµ ÑÑÐ½ÐºÑÐ¸Ð¸ ÐÐ Ð°Ð½ÐµÑÑÐµÐ·Ð¸ÑÐ¾Ð²Ð°Ð½Ð½ÑÑ Ð¼ÑÑÐµÐ¹-Ð¼Ð¾Ð´ÐµÐ»ÐµÐ¹ ÐÐ¥ Ð»Ð¸Ð½Ð¸Ð¸ YAC128 Ð¸ Ð±Ð¾Ð´ÑÑÑÐ²ÑÑÑÐ¸Ñ Ð¼ÑÑÐµÐ¹-Ð¼Ð¾Ð´ÐµÐ»ÐµÐ¹ Ð¡Ð¦Ð2 Ð»Ð¸Ð½Ð¸Ð¸ SCA2-58Q. Ð ÑÐµÐ·ÑÐ»ÑÑÐ°ÑÐµ ÑÐ¸ÑÑÐµÐ¼Ð°ÑÐ¸ÑÐµÑÐºÐ¾Ð³Ð¾ Ð²Ð²ÐµÐ´ÐµÐ½Ð¸Ñ Ð¿Ð¾Ð»Ð¾Ð¶Ð¸ÑÐµÐ»ÑÐ½Ð¾Ð³Ð¾ Ð¼Ð¾Ð´ÑÐ»ÑÑÐ¾ÑÐ° SK ÐºÐ°Ð½Ð°Ð»Ð¾Ð² ÑÐ»Ð¾ÑÐ·Ð¾ÐºÑÐ°Ð·Ð¾Ð½Ð° Ð½Ð°Ð±Ð»ÑÐ´Ð°Ð»Ð¾ÑÑ Ð²Ð¾ÑÑÑÐ°Ð½Ð¾Ð²Ð»ÐµÐ½Ð¸Ðµ ÑÐ»ÐµÐºÑÑÐ¾ÑÐ¸Ð·Ð¸Ð¾Ð»Ð¾Ð³Ð¸ÑÐµÑÐºÐ¸Ñ ÑÑÐ½ÐºÑÐ¸Ð¹ ÐÐ Ð¸ ÑÐ»ÑÑÑÐµÐ½Ð¸Ðµ ÑÐ¸Ð½Ð°Ð¿ÑÐ¸ÑÐµÑÐºÐ¾Ð¹ Ð°ÐºÑÐ¸Ð²Ð°ÑÐ¸Ð¸ ÐÐ Ð¿Ð°ÑÐ°Ð»Ð»ÐµÐ»ÑÐ½ÑÐ¼Ð¸ Ð²Ð¾Ð»Ð¾ÐºÐ½Ð°Ð¼Ð¸ Ð² ÑÐ»ÑÑÐ°Ðµ Ð¼ÑÑÐµÐ¹ ÐÐ¥ Ð¸ Ð²Ð¾ÑÑÑÐ°Ð½Ð¾Ð²Ð»ÐµÐ½Ð¸Ðµ Ð½Ð¾ÑÐ¼Ð°Ð»ÑÐ½Ð¾Ð¹ ÑÐµÐ³ÑÐ»ÑÑÐ½Ð¾ÑÑÐ¸ Ð³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð¸ Ð¸Ð¼Ð¿ÑÐ»ÑÑÐ½ÑÑ ÑÐ¸Ð³Ð½Ð°Ð»Ð¾Ð² ÐÐ Ð² ÑÐ»ÑÑÐ°Ðµ Ð¼ÑÑÐµÐ¹ Ð¡Ð¦Ð2 Ð¸ ÐÐ¥. ÐÑ Ð¿ÑÐµÐ´Ð¿Ð¾Ð»Ð°Ð³Ð°ÐµÐ¼, ÑÑÐ¾ SK ÐºÐ°Ð½Ð°Ð»Ñ Ð¼Ð¾Ð³ÑÑ Ð±ÑÑÑ ÑÐ°ÑÑÐ¼Ð¾ÑÑÐµÐ½Ñ Ð² ÐºÐ°ÑÐµÑÑÐ²Ðµ Ð²Ð¾Ð·Ð¼Ð¾Ð¶Ð½Ð¾Ð¹ Ð¼Ð¸ÑÐµÐ½Ð¸ Ð´Ð»Ñ Ð»ÐµÑÐµÐ½Ð¸Ñ ÐÐ¥ Ð¸ Ð¡Ð¦Ð2.
description: Huntingtonâs disease (HD) and several types of spinocerebellar ataxias (SCA) are the most common diseases among the neurodegenerative polyglutamine disorders. Dysfunction of cerebellar Purkinje cells (PCs) and impairment of motor functions are observed both in HD and SCA2. It is known that small conductance calcium-activated potassium channels (SK channels) are responsible for the cerebellar PC pacemaker activity. Using an extracellular single-unit recording method we evaluated electrophysiological functions of PCs of anesthetized transgenic mice-models of HD line YAC128 and awake transgenic mice-models of SCA2 line SCA2-58Q of different ages. As a result of systematic administration of positive modulator of SK channels chlorzoxazone, the restoration of electrophysiological functions of PCs and improvement of PCs synaptic activation by parallel fibers in the case of HD mice and restoration of normal firing pattern of PCs in the case of SCA2 and HD mice were observed. We propose that SK channels can be a possible target to treat HD and SCA2.