ΠΠ·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΠ΅ ΡΠ΄Π΅ΡΠ½ΠΎΠ³ΠΎ Π°ΠΊΡΠΈΠ½Π° ΠΈ NAP57/Π΄ΠΈΡΠΊΠ΅ΡΠΈΠ½Π° Ρ Π±Π΅Π»ΠΊΠ°ΠΌΠΈ, ΡΠΏΠ΅ΡΠΈΡΠΈΡΠ΅ΡΠΊΠΈ ΡΠ²ΡΠ·ΡΠ²Π°ΡΡΠΈΠΌΠΈ ΡΠ°ΡΠ΅Π»Π»ΠΈΡΠ½ΡΡ ΠΠΠ
ΠΠΈΡΡΠ΅ΡΡΠ°ΡΠΈΡ
ΠΠΎΡΡΠΎΠ²Π΅ΡΠ½ΠΎ ΠΈΠ·Π²Π΅ΡΡΠ½ΠΎ, ΡΡΠΎ ΡΠ°ΡΠΠΠ, ΠΎΠ±ΡΠ°Π·ΡΡΡΠΈΠ΅ ΡΠ΅Π½ΡΡΠΎΠΌΠ΅ΡΡ, ΠΎΠ±Π΅ΡΠΏΠ΅ΡΠΈΠ²Π°ΡΡ ΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ ΠΊΠΈΠ½Π΅ΡΠΎΡ ΠΎΡΠ°, ΠΊΠΎΡΡΠ΅ΠΊΡΠ½ΠΎΠ΅ ΡΠΏΠ°ΡΠΈΠ²Π°Π½ΠΈΠ΅ ΠΈ ΡΠ°Π·Π΄Π΅Π»Π΅Π½ΠΈΠ΅ Ρ ΡΠΎΠΌΠΎΡΠΎΠΌ Π² Ρ ΠΎΠ΄Π΅ ΠΌΠΈΡΠΎΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΈ ΠΌΠ΅ΠΉΠΎΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ Π΄Π΅Π»Π΅Π½ΠΈΠΉ ΠΊΠ»Π΅ΡΠΊΠΈ. ΠΡΠΈΡΠ΅Π½ΡΡΠΎΠΌΠ΅ΡΠ½ΡΠ΅ Π±Π»ΠΎΠΊΠΈ ΡΠ°ΡΠΠΠ Π½Π΅ΠΎΠ±Ρ ΠΎΠ΄ΠΈΠΌΡ Π΄Π»Ρ ΠΏΡΠ°Π²ΠΈΠ»ΡΠ½ΠΎΠΉ Π°ΡΡΠΎΡΠΈΠ°ΡΠΈΠΈ ΡΠ΅ΡΡΡΠΈΠ½ΡΠΊΠΈΡ Ρ ΡΠΎΠΌΠ°ΡΠΈΠ΄. Π¦Π΅Π»ΡΡ Π½Π°ΡΡΠΎΡΡΠ΅ΠΉ ΡΠ°Π±ΠΎΡΡ ΡΠ²Π»ΡΠ»ΠΎΡΡ ΠΈΠ·ΡΡΠ΅Π½ΠΈΠ΅ Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΡ Π°ΡΡΠΎΡΠΈΠΈΡΠΎΠ²Π°Π½Π½ΡΡ Ρ ΡΠ°ΡΠ΅Π»Π»ΠΈΡΠ½ΡΠΌΠΈ ΠΠΠ Π±Π΅Π»ΠΊΠΎΠ² SAF-A/hnRNP… Π§ΠΈΡΠ°ΡΡ Π΅ΡΡ >
- Π‘ΠΎΠ΄Π΅ΡΠΆΠ°Π½ΠΈΠ΅
- ΠΡΠ΄Π΅ΡΠΆΠΊΠ°
- ΠΠΈΡΠ΅ΡΠ°ΡΡΡΠ°
- ΠΡΡΠ³ΠΈΠ΅ ΡΠ°Π±ΠΎΡΡ
- ΠΠΎΠΌΠΎΡΡ Π² Π½Π°ΠΏΠΈΡΠ°Π½ΠΈΠΈ
Π‘ΠΎΠ΄Π΅ΡΠΆΠ°Π½ΠΈΠ΅
- Π‘ΠΠΠ‘ΠΠ ΠΠ‘ΠΠΠΠ¬ΠΠ£ΠΠΠ«Π₯ Π‘ΠΠΠ ΠΠ©ΠΠΠΠ
- 1. ΠΠΠΠΠΠΠΠ
- 1. 1. Π¦Π΅Π»ΠΈ ΠΈ Π·Π°Π΄Π°ΡΠΈ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ
- 1. 2. ΠΡΠ½ΠΎΠ²Π½ΡΠ΅ ΠΏΠΎΠ»ΠΎΠΆΠ΅Π½ΠΈΡ, Π²ΡΠ½ΠΎΡΠΈΠΌΡΠ΅ Π½Π° Π·Π°ΡΠΈΡΡ
- 2. ΠΠΠ’ΠΠ ΠΠ’Π£Π ΠΠ«Π ΠΠΠΠΠ
- 2. 1. ΠΡΠ³Π°Π½ΠΈΠ·Π°ΡΠΈΡ Π³Π΅Π½ΠΎΠΌΠ° ΡΡΠΊΠ°ΡΠΈΠΎΡ
- 2. 2. ΠΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΠΎΡΠ³Π°Π½ΠΈΠ·Π°ΡΠΈΠΈ ΡΠ°ΡΠ΅Π»Π»ΠΈΡΠ½ΡΡ ΠΠΠ
- 2. 3. ΠΠ΅Π»ΠΊΠΈ, ΡΠΏΠ΅ΡΠΈΡΠΈΡΠ΅ΡΠΊΠΈ ΡΠ²ΡΠ·ΡΠ²Π°ΡΡΠΈΠ΅ ΡΠ°ΡΠΠΠ
- 2. 4. ΠΠ΅ΡΠ΅ΡΠΎΡ ΡΠΎΠΌΠ°ΡΠΈΠ½
- 3. ΠΠΠ’ΠΠ ΠΠΠΠ« Π ΠΠΠ’ΠΠΠ«
- 3. 1. ΠΠΈΠΎΡ
ΠΈΠΌΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΌΠ΅ΡΠΎΠ΄Ρ
- 3. 1. 1. ΠΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ° ΡΠ΄Π΅ΡΠ½ΠΎΠ³ΠΎ ΠΌΠ°ΡΡΠΈΠΊΡΠ°
- 3. 1. 2. ΠΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ ΡΠ΄Π΅ΡΠ½ΠΎΠ³ΠΎ ΡΠΊΡΡΡΠ°ΠΊΡΠ° ΠΈΠ· ΠΊΠ»Π΅ΡΠΎΠΊ ΠΊΡΠ»ΡΡΡΡΡ HeLa
- 3. 1. 3. ΠΡΡΠΈΠ½Π½Π°Ρ ΠΎΡΠΈΡΡΠΊΠ° Π°ΠΊΡΠΈΠ½-ΡΠ²ΡΠ·ΡΠ²Π°ΡΡΠΈΡ Π±Π΅Π»ΠΊΠΎΠ² Π½Π°ΠΠΠΠ°Π·Π΅ I
- 3. 1. 4. ΠΡΡΠΈΠ½Π½Π°Ρ ΠΎΡΠΈΡΡΠΊΠ° ΡΠ΄Π΅ΡΠ½ΠΎΠ³ΠΎ Π°ΠΊΡΠΈΠ½Π°
- 3. 1. 5. ΠΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ ΠΈ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠ΅ ΡΠ°Π΄ΠΈΠΎΠ°ΠΊΡΠΈΠ²Π½ΠΎ ΠΌΠ΅ΡΠ΅Π½Π½ΡΡ ΡΡΠ°Π³ΠΌΠ΅Π½ΡΠΎΠ² Π°ΠΌΠΈΠ½ΠΎΠΊΠΈΡΠ»ΠΎΡΠ½ΠΎΠΉ ΠΏΠΎΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΠ½ΠΎΡΡΠΈ Π±Π΅Π»ΠΊΠ° SAF-A/hnRNP U
- 3. 1. 6. ΠΠ·ΡΡΠ΅Π½ΠΈΠ΅ ΡΡΠ΅ΠΏΠ΅Π½ΠΈ Π°ΡΡΠΈΠ½ΠΎΡΡΠΈ ΠΏΠ΅ΠΏΡΠΈΠ΄ΠΎΠ² ΠΊ ΡΠ΄Π΅ΡΠ½ΠΎΠΌΡ Π°ΠΊΡΠΈΠ½Ρ
- 3. 1. 1. SDS-ΡΠ»Π΅ΠΊΡΡΠΎΡΠΎΡΠ΅Π· ΠΈ ΠΎΠΊΡΠ°ΡΠΈΠ²Π°Π½ΠΈΠ΅ Π³Π΅Π»Π΅ΠΉ
- 3. 2. ΠΠΎΠ»Π΅ΠΊΡΠ»ΡΡΠ½ΡΠ΅ ΠΌΠ΅ΡΠΎΠ΄Ρ
- 3. 2. 1. ΠΠ»Π°Π·ΠΌΠΈΠ΄Ρ
- 3. 2. 2. ΠΡΠ΄Π΅Π»Π΅Π½ΠΈΠ΅ ΠΏΠ»Π°Π·ΠΌΠΈΠ΄Π½ΠΎΠΉ ΠΠΠ, ΡΠ»Π΅ΠΊΡΡΠΎΡΠΎΡΠ΅Π· ΠΠΠ
- 3. 2. 3. ΠΠΎΠ»ΠΈΠΌΠ΅ΡΠ°Π·Π½Π°Ρ ΡΠ΅ΠΏΠ½Π°Ρ ΡΠ΅Π°ΠΊΡΠΈΡ (ΠΠ¦Π )
- 3. 2. 4. ΠΡΠ°ΠΉΠΌΠ΅ΡΡ
- 3. 2. 5. ΠΠ»ΠΎΠ½ΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ ΡΡΠ°Π³ΠΌΠ΅Π½ΡΠΎΠ² ΠΠΠ
- 3. 3. ΠΠΌΠΌΡΠ½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΌΠ΅ΡΠΎΠ΄Ρ
- 3. 1. ΠΠΈΠΎΡ
ΠΈΠΌΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΌΠ΅ΡΠΎΠ΄Ρ
- 3. 3,1. ΠΠ½ΡΠΈΡΠ΅Π»Π°
- 3. 3. 2. ΠΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ ΠΏΠΎΠ»ΠΈΡΠΎΠ½Π°Π»ΡΠ½ΡΡ Π°Π½ΡΠΈΡΠ΅Π»
- 3. 3. 3. ΠΠΌΠΌΡΠ½ΠΎΠΏΡΠ΅ΡΠΈΠΏΠΈΡΠ°ΡΠΈΡ
- 3. 3. 4. ΠΠΌΠΌΡΠ½ΠΎΠ±Π»ΠΎΡΠΈΠ½Π³
- 3. 4. Π¦ΠΈΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΠΌΠ΅ΡΠΎΠ΄Ρ
- 3. 4. 1. ΠΡΠ»ΡΡΠΈΠ²ΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ ΠΊΠ»Π΅ΡΠΎΠΊ
- 3. 4. 2. ΠΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠ΅ Π΄ΠΈΠΎΡΠΈΠΎΠ±ΠΈΡ-ΡΡΠΊΡΠΈΠ½Π³ΡΠΈΠ΄ΠΈΠ»ΠΏΡΠΎΠΏΠΈΠΎΠ½Π°ΡΠ°
- 3. 4. 3. Π’ΡΠ°Π½ΡΡΠ΅ΠΊΡΠΈΡ ΠΊΠ»Π΅ΡΠΎΠΊ ΠΊΡΠ»ΡΡΡΡΡ
- 3. 4. 4. Π‘ΠΈΠ½Ρ ΡΠΎΠ½ΠΈΠ·Π°ΡΠΈΡ ΠΊΠ»Π΅ΡΠΎΠΊ
- 3. 4. 5. ΠΠΌΠΌΡΠ½ΠΎΡΠ»ΡΠΎΡΠ΅ΡΡΠ΅Π½ΡΠΈΡ
- 3. 4. 6. ΠΠΈΠΊΡΠΎΠΈΠ½ΡΠ΅ΠΊΡΠΈΠΈ
- 3. 4. 7. ΠΡΡΠ²Π»Π΅Π½ΠΈΠ΅ ΡΠ°ΠΉΡΠΎΠ² ΡΡΠ°Π½ΡΠΊΡΠΈΠΏΡΠΈΠΈ
- 3. 5. ΠΠ΅ΡΠΎΠ΄Ρ ΠΊΠΎΠΌΠΏΡΡΡΠ΅ΡΠ½ΠΎΠ³ΠΎ Π°Π½Π°Π»ΠΈΠ·Π°
- Π ΠΠΠ£ΠΠ¬Π’ΠΠ’Π«
- 4. 1. ΠΠΎΠ»ΡΡΠ΅Π½ΠΈΠ΅ ΠΈ ΡΠ΅ΡΡΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ ΠΏΠΎΠ»ΠΈΠΊΠ»ΠΎΠ½Π°Π»ΡΠ½ΡΡ Π°Π½ΡΠΈΡΠ΅Π» ΠΏΡΠΎΡΠΈΠ² Π±Π΅Π»ΠΊΠ° NAP5 7/D yskerin
- 4. 2. ΠΠΎΠΊΠ°Π»ΠΈΠ·Π°ΡΠΈΡ Π±Π΅Π»ΠΊΠΎΠ² Π¨Π Π 140 ΠΈ NAP57/Dyskerin
- 4. 3. ΠΠ½ΡΡΡΠΈΡΠ΄Π΅ΡΠ½Π°Ρ Π»ΠΎΠΊΠ°Π»ΠΈΠ·Π°ΡΠΈΡ Π±Π΅Π»ΠΊΠ° NAP57/Dyskerin
- 4. 4. ΠΠΎΠΊΠ°Π»ΠΈΠ·Π°ΡΠΈΡ Π±Π΅Π»ΠΊΠ° NAP57 ΠΈ Π ΠΠ-Π³Π΅Π»ΠΈΠΊΠ°Π·Ρ Ρ68 ΡΠ΅ΠΌΠ΅ΠΉΡΡΠ²Π° DEAD
- 4. 5. ΠΠΎΠΌΠ΅Π½Π½ΡΠΉ Π°Π½Π°Π»ΠΈΠ· ΠΏΠ΅ΡΠ²ΠΈΡΠ½ΠΎΠΉ ΠΏΠΎΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΠ½ΠΎΡΡΠΈ Π±Π΅Π»ΠΊΠ° SAF-A/hnRNPU
- 4. 6. ΠΠ·ΡΡΠ΅Π½ΠΈΠ΅ ΡΡΠ½ΠΊΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠΉ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΠΈ Ρ Π°ΡΠ°ΠΊΡΠ΅ΡΠ° Π»ΠΎΠΊΠ°Π»ΠΈΠ·Π°ΡΠΈΠΈ ΠΎΡΠ΄Π΅Π»ΡΠ½ΡΡ Π΄ΠΎΠΌΠ΅Π½ΠΎΠ² Π±Π΅Π»ΠΊΠ° SAF-A, ΠΌΠ΅ΡΠ΅Π½Π½ΡΡ GFP
- 4. 7. ΠΠ΅Π»ΠΎΠΊ SAF-A Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΡΠ΅Ρ Ρ ΡΠ΄Π΅ΡΠ½ΡΠΌ Π -Π°ΠΊΡΠΈΠ½ΠΎΠΌ in vitro ΠΈ in vivo
- 4. 8. ΠΠΎΠΊΠ°Π»ΠΈΠ·Π°ΡΠΈΡ Π±Π΅Π»ΠΊΠ° SAF-A ΠΈ ΡΠ΄Π΅ΡΠ½ΠΎΠ³ΠΎ Π°ΠΊΡΠΈΠ½Π° Π² ΠΈΠ½ΡΠ΅ΡΡΠ°Π·Π½ΡΡ ΠΈ ΠΌΠΈΡΠΎΡΠΈΡΠ΅ΡΠΊΠΈΡ ΠΊΠ»Π΅ΡΠΊΠ°Ρ
- 4. 9. ΠΠ΅Π»ΠΎΠΊ SAF-A Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΡΠ΅Ρ Ρ Π -Π°ΠΊΡΠΈΠ½ΠΎΠΌ Π² ΡΠ΄ΡΠ΅ ΡΠ΅ΡΠ΅Π· Π‘-ΠΊΠΎΠ½ΡΠ΅Π²ΠΎΠΉ Π΄ΠΎΠΌΠ΅Π½ Π±Π΅Π»ΠΊΠ°
- 4. 10. ΠΠ΅Π»ΠΎΠΊ SAF-A Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΡΠ΅Ρ Ρ Π -Π°ΠΊΡΠΈΠ½ΠΎΠΌ Π² ΡΠ΄ΡΠ΅ ΡΠ΅ΡΠ΅Π· ΠΏΡΡΠΈ Π°ΠΌΠΈΠ½ΠΎΠΊΠΈΡΠ»ΠΎΡΠ½ΡΡ ΠΏΠΎΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΠ½ΠΎΡΡΡ QRTQK Π½Π° Π‘-ΠΊΠΎΠ½ΡΠ΅
- 4. 11. ΠΠΎΠΌΠΏΠ»Π΅ΠΊΡ SAF-A Ρ Π -Π°ΠΊΡΠΈΠ½ΠΎΠΌ Π½Π΅ΠΎΠ±Ρ ΠΎΠ΄ΠΈΠΌ Π΄Π»Ρ ΡΡΠ°Π½ΡΠΊΡΠΈΠΏΡΠΈΠΈ Π ΠΠ-ΠΏΠΎΠ»ΠΈΠΌΠ΅ΡΠ°Π·ΠΎΠΉII
- 5. ΠΠΠ‘Π£ΠΠΠΠΠΠ Π ΠΠΠ£ΠΠ¬Π’ΠΠ’ΠΠ
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