title: ÐÑÐ¿Ð¾Ð»ÑÐ·Ð¾Ð²Ð°Ð½Ð¸Ðµ Ð¼ÐµÑÐ¾Ð´Ð° Ð½ÐµÐ¿ÑÑÐ¼Ð¾Ð¹ Ð¸Ð¼Ð¼ÑÐ½Ð¾ÑÐ»ÑÐ¾ÑÐµÑÑÐµÐ½ÑÐ¸Ð¸ Ð´Ð»Ñ ÑÑÐ¾ÑÐ½ÐµÐ½Ð¸Ñ Ð´Ð¸Ð°Ð³Ð½Ð¾Ð·Ð¾Ð² Ð¿ÑÐ¸ Ð°ÑÐ°ÐºÑÐ¸Ð¸-ÑÐµÐ»ÐµÐ°Ð½Ð³Ð¸ÑÐºÑÐ°Ð·Ð¸Ð¸ Ð¸ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ðµ Ð¡ÐµÐºÐºÐµÐ»Ñ
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 16.04.01 - Ð¢ÐµÑ Ð½Ð¸ÑÐµÑÐºÐ°Ñ ÑÐ¸Ð·Ð¸ÐºÐ° ; 16.04.01_10 - ÐÐµÐ´Ð¸ÑÐ¸Ð½ÑÐºÐ°Ñ Ð±Ð¸Ð¾ÑÐµÑ Ð½Ð¾Ð»Ð¾Ð³Ð¸Ñ
titleType: Subtitle
Creators: name: ÐÐ¾ÑÑÑÐº, ÐÐ¼Ð¸ÑÑÐ¸Ð¹
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°ÑÐ°ÐºÑÐ¸Ñ-ÑÐµÐ»ÐµÐ°Ð½Ð³Ð¸ÑÐºÑÐ°Ð·Ð¸Ð¸Ñ
ÑÐ¸Ð½Ð´ÑÐ¾Ð¼ Ð¡ÐµÐºÐºÐµÐ»Ñ
Ð½ÐµÐ¿ÑÑÐ¼Ð°Ñ Ð¸Ð¼Ð¼ÑÐ½Ð¾ÑÐ»ÑÐ¾ÑÐµÑÑÐµÐ½ÑÐ¸Ñ
Ð¿ÑÐµÐ¶Ð´ÐµÐ²ÑÐµÐ¼ÐµÐ½Ð½Ð¾Ðµ ÑÑÐ°ÑÐµÐ½Ð¸Ðµ
Ð³ÐµÐ½ÐµÑÐ¸ÑÐµÑÐºÐ¸Ðµ Ð¼ÑÑÐ°ÑÐ¸Ð¸
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description: ÐÑÐ°ÐºÑÐ¸Ñ-ÑÐµÐ»ÐµÐ°Ð½Ð³Ð¸ÑÐºÑÐ°Ð·Ð¸Ñ (ÐÐ¢) â ÑÑÐ¶ÑÐ»Ð¾Ðµ Ð¿ÑÐ¾Ð³ÑÐµÑÑÐ¸ÑÑÑÑÐµÐµ Ð¼ÑÐ»ÑÑÐ¸ÑÐ¸ÑÑÐµÐ¼Ð½Ð¾ÐµÐ½ÐµÐ¹ÑÐ¾Ð´ÐµÐ³ÐµÐ½ÐµÑÐ°ÑÐ¸Ð²Ð½Ð¾Ðµ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ðµ Ñ Ð¿ÑÐ¸Ð·Ð½Ð°ÐºÐ°Ð¼Ð¸ Ð¿ÑÐµÐ¶Ð´ÐµÐ²ÑÐµÐ¼ÐµÐ½Ð½Ð¾Ð³Ð¾ ÑÑÐ°ÑÐµÐ½Ð¸Ñ Ð¸ ÑÐµÐ·ÐºÐ¸Ð¼ Ð¿Ð¾Ð²ÑÑÐµÐ½Ð½ÑÐ¼ ÑÐ¸ÑÐºÐ¾Ð¼ Ð¾Ð±ÑÐ°Ð·Ð¾Ð²Ð°Ð½Ð¸Ñ Ð¾Ð¿ÑÑÐ¾Ð»ÐµÐ¹, ÐºÐ¾ÑÐ¾ÑÐ¾Ðµ Ð½Ð°ÑÐ»ÐµÐ´ÑÐµÑÑÑ Ð¿Ð¾ Ð°ÑÑÐ¾ÑÐ¾Ð¼Ð½Ð¾-ÑÐµÑÐµÑÑÐ¸Ð²Ð½Ð¾Ð¼Ñ ÑÐ¸Ð¿Ñ. ÐÑÐ¸ÑÐ¸Ð½Ð¾Ð¹ ÑÑÐ¾Ð³Ð¾ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ñ ÑÐ²Ð»ÑÑÑÑÑ Ð¼ÑÑÐ°ÑÐ¸Ð¸ Ð² Ð³ÐµÐ½Ðµ ATM, Ð»Ð¾ÐºÐ°Ð»Ð¸Ð·Ð¾Ð²Ð°Ð½Ð½Ð¾Ð¼ Ð² 11 ÑÑÐ¾Ð¼Ð¾ÑÐ¾Ð¼Ðµ (11q23), Ð¸Ð¼ÐµÑÑÐµÐ¼ ÑÐ°Ð·Ð¼ÐµÑ 150 Ñ.Ð¿.Ð½. Ð¸ ÑÐ¾Ð´ÐµÑÐ¶Ð°ÑÐµÐ¼ 66 ÑÐºÐ·Ð¾Ð½Ð¾Ð²[3]. ÐÑÑÐ¾ÐºÐ¾Ð¼Ñ ÑÐ¸ÑÐºÑ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð¾Ð¿ÑÑÐ¾Ð»ÐµÐ¹ ÑÐ°ÐºÐ¶Ðµ Ð¿Ð¾Ð´Ð²ÐµÑÐ¶ÐµÐ½Ñ Ð³ÐµÑÐµÑÐ¾Ð·Ð¸Ð³Ð¾ÑÐ½ÑÐµ Ð½Ð¾ÑÐ¸ÑÐµÐ»Ð¸. ÐÐ»Ð¸Ð½Ð¸ÑÐµÑÐºÐ°Ñ ÐºÐ°ÑÑÐ¸Ð½Ð° Ð¿ÑÐ¸ ÐÐ¢ Ð¿Ð¾Ð»Ð¸Ð¼Ð¾ÑÑÐ½Ð° Ð¸ Ð¿ÑÐ¸ Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐµ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ñ Ð¼Ð¾Ð³ÑÑ Ð²Ð¾Ð·Ð½Ð¸ÐºÐ½ÑÑÑ ÑÑÑÐ´Ð½Ð¾ÑÑÐ¸. Ð£ÑÐ¸ÑÑÐ²Ð°Ñ, ÑÑÐ¾ Ð¼Ð¾Ð»ÐµÐºÑÐ»ÑÑÐ½Ð¾-Ð³ÐµÐ½ÐµÑÐ¸ÑÐµÑÐºÐ°Ñ Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐ° Ð·Ð°ÑÑÑÐ´Ð½ÐµÐ½Ð° Ð¸Ð·-Ð·Ð° Ð±Ð¾Ð»ÑÑÐ¾Ð³Ð¾ ÐºÐ¾Ð»Ð¸ÑÐµÑÑÐ²Ð° Ð¼ÑÑÐ°ÑÐ¸Ð¹, ÑÐ°Ð·Ð¼ÐµÑÐ° Ð¸ Ð²ÑÑÐ¾ÐºÐ¾Ð¹ ÑÑÐµÐ¿ÐµÐ½Ð¸ Ð¿Ð¾Ð»Ð¸Ð¼Ð¾ÑÑÐ¸Ð·Ð¼Ð° Ð³ÐµÐ½Ð° ATM, Ð¸ÑÑÐ»ÐµÐ´Ð¾Ð²Ð°Ð½Ð¸Ñ ÐºÐ»ÐµÑÐ¾ÑÐ½ÑÑ Ð¾ÑÐ¾Ð±ÐµÐ½Ð½Ð¾ÑÑÐµÐ¹ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ ÐÐ¢ Ð¸Ð¼ÐµÑÑ Ð±Ð¾Ð»ÑÑÐ¾Ðµ Ð·Ð½Ð°ÑÐµÐ½Ð¸Ðµ Ð´Ð»Ñ ÑÐ°Ð·ÑÐ°Ð±Ð¾ÑÐºÐ¸ Ð±Ð¾Ð»ÐµÐµ ÑÐ¾ÑÐ½ÑÑ Ð¼ÐµÑÐ¾Ð´Ð¾Ð² Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐ¸ Ð´Ð°Ð½Ð½Ð¾Ð³Ð¾ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ñ. Ð¡Ð¸Ð½Ð´ÑÐ¾Ð¼ CÐµÐºÐºÐµÐ»Ñ, Ð¸Ð»Ð¸ Ð¼Ð¸ÐºÑÐ¾ÑÐµÑÐ°Ð»ÑÐ½Ð°Ñ Ð¿ÐµÑÐ²Ð¸ÑÐ½Ð°Ñ ÐºÐ°ÑÐ»Ð¸ÐºÐ¾Ð²Ð¾ÑÑÑ ÑÐ²Ð»ÑÐµÑÑÑ ÑÑÐµÐ·Ð²ÑÑÐ°Ð¹Ð½Ð¾ ÑÐµÐ´ÐºÐ¸Ð¼ Ð²ÑÐ¾Ð¶Ð´ÐµÐ½Ð½ÑÐ¼ Ð½Ð°Ð½Ð¾ÑÐ¾Ð¼Ð½ÑÐ¼ ÑÐ°ÑÑÑÑÐ¾Ð¹ÑÑÐ²Ð¾Ð¼. ÐÐ°ÑÐ»ÐµÐ´Ð¾Ð²Ð°Ð½Ð¸Ðµ Ð°ÑÑÐ¾ÑÐ¾Ð¼Ð½Ð¾-ÑÐµÑÐµÑÑÐ¸Ð²Ð½Ð¾Ðµ. Ð¡Ð¸Ð½Ð´ÑÐ¾Ð¼ Ð¡ÐµÐºÐºÐµÐ»Ñ ÑÐ°ÑÐ°ÐºÑÐµÑÐ¸Ð·ÑÐµÑÑÑ Ð·Ð°Ð´ÐµÑÐ¶ÐºÐ¾Ð¹ Ð²Ð½ÑÑÑÐ¸ÑÑÑÐ¾Ð±Ð½Ð¾Ð³Ð¾ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð¸ Ð¿Ð¾ÑÐ»ÐµÑÐ¾Ð´Ð¾Ð²Ð¾Ð¹ ÐºÐ°ÑÐ»Ð¸ÐºÐ¾Ð²Ð¾ÑÑÑÑ Ñ Ð¼Ð°Ð»ÐµÐ½ÑÐºÐ¾Ð¹ Ð³Ð¾Ð»Ð¾Ð²Ð¾Ð¹, ÑÐ·ÐºÐ¸Ð¼ Ð¿ÑÐ¸ÑÐµÐ¿Ð¾Ð´Ð¾Ð±Ð½ÑÐ¼ Ð»Ð¸ÑÐ¾Ð¼ Ñ ÐºÐ»ÑÐ²Ð¾Ð¿Ð¾Ð´Ð¾Ð±Ð½ÑÐ¼ Ð½Ð¾ÑÐ¾Ð¼, Ð±Ð¾Ð»ÑÑÐ¸Ð¼Ð¸ Ð³Ð»Ð°Ð·Ð°Ð¼Ð¸ Ñ Ð½Ð°ÐºÐ»Ð¾Ð½ÐµÐ½Ð½ÑÐ¼Ð¸ Ð²Ð½Ð¸Ð· Ð³Ð»Ð°Ð·Ð½ÑÐ¼Ð¸ ÑÐµÐ»ÑÐ¼Ð¸, Ð½Ð¸Ð·ÐºÐ¾ Ð¿Ð¾ÑÐ°Ð¶ÐµÐ½Ð½ÑÐ¼Ð¸ ÑÑÐ°Ð¼Ð¸, ÑÐºÐ¾ÑÐ¾ÑÐµÐ½Ð½Ð¾Ð¹ Ð½Ð¸Ð¶Ð½ÐµÐ¹ ÑÐµÐ»ÑÑÑÑÑ (Ð¼Ð¸ÐºÑÐ¾Ð³Ð½Ð°ÑÐ¸Ñ) Ð¸ Ð·Ð°Ð´ÐµÑÐ¶ÐºÐ¾Ð¹ ÑÐ¼ÑÑÐ²ÐµÐ½Ð½Ð¾Ð³Ð¾ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ. ÐÑÐ¼ÐµÑÐµÐ½Ð° ÑÐ°ÐºÐ¶Ðµ Ð¿Ð°Ð½ÑÐ¸ÑÐ¾Ð¿ÐµÐ½Ð¸Ñ Ð¸ Ð³Ð¸Ð¿Ð¾Ð³Ð°Ð¼Ð¼Ð°Ð³Ð»Ð¾Ð±ÑÐ»Ð¸Ð½ÐµÐ¼Ð¸Ñ. Ð Ð½Ð°ÑÑÐ¾ÑÑÐµÐ¼Ñ Ð²ÑÐµÐ¼ÐµÐ½Ð¸ Ñ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð° Ð¡ÐµÐºÐºÐµÐ»Ñ Ð¾Ð¿Ð¸ÑÐ°Ð½Ñ ÑÐµÑÑÑ Ð³ÑÑÐ¿Ð¿ ÐºÐ¾Ð¼Ð¿Ð»ÐµÐ¼ÐµÐ½ÑÐ°ÑÐ¸Ð¸ ÑÐ¾ ÐµÑÑÑ Ð¡Ð¸Ð½Ð´ÑÐ¾Ð¼ Ð¡ÐµÐºÐºÐµÐ»Ñ ÑÐ°Ð·Ð²Ð¸Ð²Ð°ÐµÑÑÑ Ð¿ÑÐ¸ Ð½Ð°Ð»Ð¸ÑÐ¸Ð¸ Ð¼ÑÑÐ°ÑÐ¸Ð¹ Ð² ÑÐµÑÑÐ¸ ÑÐ°Ð·Ð»Ð¸ÑÐ½ÑÑ Ð³ÐµÐ½Ð°Ñ: ATR, RBBP8, CENPJ, CEP152, CEP63, NIN [12]. 4 ÐÐ°Ð¸Ð±Ð¾Ð»ÐµÐµ ÑÐ°ÑÑÐ¾ Ð²ÑÑÑÐµÑÐ°ÑÑÐ¸Ð¼ÑÑ Ð¸ Ð¿Ð¾Ð´ÑÐ¾Ð±Ð½Ð¾ Ð¾Ð¿Ð¸ÑÐ°Ð½Ð½ÑÐ¼ ÑÐ²Ð»ÑÐµÑÑÑ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼ Ð¡ÐµÐºÐºÐµÐ»Ñ 1 ÑÐ¸Ð¿Ð°, Ð²Ð¾Ð·Ð½Ð¸ÐºÐ°ÑÑÐ¸Ð¹ Ð¿ÑÐ¸ Ð¾ÑÑÑÑÑÑÐ²Ð¸Ð¸ Ð°ÐºÑÐ¸Ð²Ð½Ð¾Ð¹ ÑÐ¾ÑÐ¼Ñ Ð¿ÑÐ¾ÑÐµÐ¸Ð½ÐºÐ¸Ð½Ð°Ð·ÑATR[12]. Ð Ð½ÐµÐºÐ¾ÑÐ¾ÑÑÑ ÑÐ»ÑÑÐ°ÑÑ ÑÑÐ¾Ð´Ð½ÑÐµ ÑÐ¸Ð¼Ð¿ÑÐ¾Ð¼Ñ Ð¼Ð¾Ð³ÑÑ Ð±ÑÑÑ Ð¾Ð¿Ð¸ÑÐ°Ð½Ñ Ñ Ð¿Ð°ÑÐ¸ÐµÐ½ÑÐ¾Ð² Ñ ÐÐµÐ¹Ð¼Ð¸Ð³ÐµÐ½ÑÐºÐ¸Ð¼ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð¾Ð¼ Ð»Ð¾Ð¼ÐºÐ¾ÑÑÐ¸ ÑÑÐ¾Ð¼Ð¾ÑÐ¾Ð¼ (NBS). ÐÐ·-Ð·Ð° ÑÐ°ÑÑÐ¾ Ð¿ÐµÑÐµÐºÑÑÐ²Ð°ÑÑÐ¸ÑÑÑ ÐºÐ»Ð¸Ð½Ð¸ÑÐµÑÐºÐ¸Ñ Ð¿ÑÐ¸Ð·Ð½Ð°ÐºÐ¾Ð², Ð¾ÑÐ¾Ð±ÐµÐ½Ð½Ð¾ Ð½Ð° ÑÐ°Ð½Ð½Ð¸Ñ ÑÑÐ°Ð¿Ð°Ñ ÑÐ°Ð·Ð²Ð¸ÑÐ¸Ñ Ð·Ð°Ð±Ð¾Ð»ÐµÐ²Ð°Ð½Ð¸Ñ, Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐ° ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð° Ð¡ÐµÐºÐºÐµÐ»Ñ ÑÐ°ÐºÐ¶Ðµ Ð·Ð°ÑÑÑÐ´Ð½ÐµÐ½Ð°. Ð¢Ð°Ðº ÐºÐ°Ðº Ð³ÐµÐ½Ñ ÐÐ¢Ð Ð¸ ATR ÑÐ²Ð»ÑÑÑÑÑ Ð±Ð¾Ð»ÑÑÐ¸Ð¼Ð¸ Ð¿Ð¾ ÑÐ°Ð·Ð¼ÐµÑÑ, Ð¾ÑÐ»Ð¸ÑÐ°ÑÑÑÑ Ð²ÑÑÐ°Ð¶ÐµÐ½Ð½ÑÐ¼ Ð¿Ð¾Ð»Ð¸Ð¼Ð¾ÑÑÐ¸Ð·Ð¼Ð¾Ð¼, Ð° ÑÐ¿ÐµÐºÑÑ Ð¼ÑÑÐ°ÑÐ¸Ð¹ Ð´Ð¾ÑÑÐ°ÑÐ¾ÑÐ½Ð¾ ÑÐ¸ÑÐ¾Ðº (Ð±Ð¾Ð»ÑÑÐ¸Ð½ÑÑÐ²Ð¾ Ð±Ð¾Ð»ÑÐ½ÑÑ ÑÐ²Ð»ÑÑÑÑÑ ÐºÐ¾Ð¼Ð¿Ð°ÑÐ½Ð´Ð°Ð¼Ð¸), ÑÐ¾ Ð¼Ð¾Ð»ÐµÐºÑÐ»ÑÑÐ½Ð¾-Ð³ÐµÐ½ÐµÑÐ¸ÑÐµÑÐºÐ°Ñ Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐ° ÑÑÐ¸Ñ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð¾Ð² Ð·Ð°ÑÑÑÐ´Ð½ÐµÐ½Ð°. ÐÐ»Ñ Ð±ÑÑÑÑÐ¾Ð³Ð¾ ÑÑÐ¾ÑÐ½ÐµÐ½Ð¸Ñ ÐºÐ»Ð¸Ð½Ð¸ÑÐµÑÐºÐ¾Ð³Ð¾ Ð´Ð¸Ð°Ð³Ð½Ð¾Ð·Ð° Ð°ÑÐ°ÐºÑÐ¸Ð¸-ÑÐµÐ»ÐµÐ°Ð½Ð³Ð¸ÑÐºÑÐ°Ð·Ð¸Ð¸ Ð¸ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð° Ð¡ÐµÐºÐºÐºÐµÐ»Ñ 1 ÑÐ¸Ð¿Ð° Ð² Ð»Ð°Ð±Ð¾ÑÐ°ÑÐ¾ÑÐ¸Ð¸ ÑÐ°Ð´Ð¸Ð°ÑÐ¸Ð¾Ð½Ð½Ð¾Ð¹ ÑÐ¸ÑÐ¾Ð»Ð¾Ð³Ð¸Ð¸ ÐÐÐ¦ Ð ÐÐ Ð±ÑÐ» ÑÐ°Ð·ÑÐ°Ð±Ð¾ÑÐ°Ð½ Ð¼ÐµÑÐ¾Ð´ Ð´Ð¸Ð°Ð³Ð½Ð¾ÑÑÐ¸ÐºÐ¸ Ð½Ð° ÐºÐ»ÐµÑÐ¾ÑÐ½Ð¾Ð¼ ÑÑÐ¾Ð²Ð½Ðµ Ñ Ð¿ÑÐ¸Ð¼ÐµÐ½ÐµÐ½Ð¸ÐµÐ¼ Ð½ÐµÐ¿ÑÑÐ¼Ð¾Ð¹ Ð¸Ð¼Ð¼ÑÐ½Ð¾ÑÐ»ÑÐ¾ÑÐµÑÑÐµÐ½ÑÐ¸Ð¸[38] . ÐÐ°Ð³Ð¸ÑÑÐµÑÑÐºÐ¾Ðµ Ð¸ÑÑÐ»ÐµÐ´Ð¾Ð²Ð°Ð½Ð¸Ðµ ÐÐ¾ÑÑÑÐºÐ° Ð.Ð. Ð¿Ð¾ÑÐ²ÑÑÐµÐ½Ð¾ ÑÑÐ¾ÑÐ½ÐµÐ½Ð¸Ñ Ð´Ð¸Ð°Ð³Ð½Ð¾Ð·Ð° Ð´Ð²ÑÑ Ð²Ð½Ð¾Ð²Ñ Ð¾Ð¿Ð¸ÑÐ°Ð½Ð½ÑÑ ÐºÐ»Ð¸Ð½Ð¸ÑÐµÑÐºÐ¸Ñ ÑÐ»ÑÑÐ°ÐµÐ² â Ð°ÑÐ°ÐºÑÐ¸Ð¸-ÑÐµÐ»ÐµÐ°Ð½Ð³Ð¸ÑÐºÑÐ°Ð·Ð¸Ð¸ (Ð¼Ð°Ð»ÑÑÐ¸Ðº 15 Ð»ÐµÑ) Ð¸ ÑÐ¸Ð½Ð´ÑÐ¾Ð¼Ð° Ð¡ÐµÐºÐºÐµÐ»Ñ (Ð¼Ð°Ð»ÑÑÐ¸Ðº 6 Ð¼ÐµÑÑÑÐµÐ²).
description: Ataxia-telangiectasia (at) â a severe progressive multisystemic degenerative disease with signs of premature aging and a sharp increased risk of tumors, which is inherited by autosomal recessive type. The cause of this disease is mutations in the ATM gene localized in 11 chromosomes (11q23), having a size of 150 t. n. and containing 66 exons[3]. Heterozygous carriers are also at high risk of developing tumors. The clinical picture in at is polymorphic and in the diagnosis of the disease there may be difficulties. Given that molecular genetic diagnosis is difficult due to the large number of mutations, the size and high degree of polymorphism of the ATM gene, studies of cellular characteristics of patients with at are of great importance for the development of more accurate methods of diagnosis of this disease. Seckel syndrome, or microcephalic primary dwarfism, is an extremely rare congenital nanosomal disorder. Inheritance is autosomal recessive. Seckel syndrome is characterized by intrauterine growth retardation and postpartum dwarfism with a small head, a narrow bird-like face with a beak-like nose, large eyes with tilted down eye slits, low-set ears, a shortened lower jaw (micrognatia) and mental retardation. Pancytopenia and hypogammaglobulinemia were also noted. To date, seckel syndrome describes six groups of complementation that is, Seckel Syndrome develops in the presence of mutations in six different genes: ATR, RBBP8, CENPJ, CEP152, CEP63, NIN [12]. The most common and described in detail is type 1 Seckel syndrome, which occurs in the absence of an active form of protein kinase Tr [12]. In some cases, similar symptoms may be described in patients with Nijmigen chromosome fragility syndrome (NBS). Because of the often overlapping clinical signs, especially in the early stages of the disease, the diagnosis of Seckel syndrome is also difficult. Since the genes of ATM and ATR are large in size, characterized by 6 pronounced polymorphism, and the mutation spectrum is wide enough (most patients are compounds), the molecular genetic diagnosis of these syndromes is difficult. For a quick clarification of the clinical diagnosis of ataxia telangiectasia and Cecchele syndrome type 1 in the laboratory of radiation Cytology ISC RAS we have developed the method of diagnosis at the cellular level using indirect immunofluorescence[38]. Master's research Matruka D. A. is devoted to clarify the diagnosis of two newly described clinical cases of ataxia telangiectasia (boy 15 years) and the syndrome of Sekela (boy 6 months).