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Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis
�깮�솕�븰�떎�뒿
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CONTENTS
INTRODUCTION
PATHOGENIC MECHANISM
MOTOR NEURON VULNERABILITY
FROM MOLECULAR TO CLINICAL FEATURES
CONCLUSION
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INTRODUCTION
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Introduction
ALS (Amyotrophic lateral sclerosis)
- ALS ���?
- a.k.a 猷④쾶由� 蹂�
- 10留뚮챸 以� 2紐� 瑗대줈 諛쒖깮 (Kii 諛섎룄��� 愿뚯뿉�꽌 �넂��� 鍮덈룄)
- 二쇰줈 55-60�꽭�뿉 諛쒕퀝, �깮議닿린媛� �빟 3�뀈
- �뿬�꽦蹂대떎 �궓�꽦�씠 諛쒕퀝 鍮덈룄媛� �넂�떎.
- ALS�쓽 遺꾨쪟
- 媛�議깆쟻 ALS(familial ALS, FALS)
- �궛諛쒖쟻 ALS(sporadic ALS ,SALS)
Amyotrophic Lateral Sclerosis
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Introduction
ALS (Amyotrophic lateral sclerosis)
- ALS ���?
- �떊寃쏀뙆愿댁쭏蹂�(neurodegenerative disorder)
- �듅�엳 �슫�룞�떊寃쎌꽭�룷留� �꽑�깮�쟻�쑝濡� �궗硫명븯�뒗 吏덊솚
- ����뇤 �뵾吏덉쓽 �쐞�슫�룞�떊寃쎌꽭�룷(upper motor neuron,
�긽�쐞�슫�룞�떊寃쎌꽭�룷)��� �뇤媛� 諛� 泥숈닔�쓽 �븘�옒�슫�룞�떊寃쎌꽭�룷
(lower motor neuron) 紐⑤몢媛� �젏李⑥쟻�쑝濡� �뙆愿�
Amyotrophic Lateral Scl���(�깮�왂)
�몺 �쟾泥댁쟻�씤 蹂��솕
�몼 誘몄꽭�븳 蹂��솕
�몺 �쑀�쟾�옄�쓽 polymorphism�씠 諛쒓껄
�몼 �쑀�쟾�옄 �닔�쓽 �떎�뼇�꽦
�몾 �옞�옱�쟻�씤 ALS 誘쇨컧�꽦 �쐞移섎�� 諛쒓껄
�몿 Ataxin-2�뿉�꽌 以묎컙 湲몄씠�쓽 polyglutamine �솗�옣�씠 諛쒓껄
2. Oxidative stress
�몺 SALS, SOD1-related FALS�쓽 二쎌�� 議곗쭅�꽭�룷瑜� 遺꾩꽍�븳 寃곌낵
�몼 mouse model
�몾 TAR DNA-binding protein (TDP-43)-related ALS �룎�뿰蹂��씠�쓽
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zymes PON1?3,
- survival motor neuron genes SMN1 and SMN2
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Genetic Factors
Genetic factor;�쑀�쟾�쟻 �슂�냼
�쑀�쟾�쟻 �슂�냼(Genetic factors)
- SALS �솚�옄�쓽 �쑀�쟾�옄 遺꾩꽍
�몾 �옞�옱�쟻�씤 ALS 誘쇨컧�꽦 �쐞移섎�� 諛쒓껄
- FGGY
- inositol 1,4,5-trisphosphate receptor type 2 (ITPR2)
- dipeptidyl-peptidase 6 (DPP6)
�몿 Ataxin-2�뿉�꽌 以묎컙 湲몄씠�쓽 polyglutamine �솗�옣�씠 諛쒓껄
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2. Oxidative stress
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Oxidative Stress
Oxidative stress;�궛�솕�쟻 �뒪�듃�젅�뒪
- �궛�솕�쟻 �뒪�듃�젅�뒪�쓽 異뺤쟻 �넂 吏덈퀝�쑀諛� 蹂��씠�쓽 議댁옱�굹, �돱�윴�쓽
二쎌쓬�쑝濡� �걹�굹�뒗 �꽭�룷�넀�긽�쓽 �븙�닚�솚, 以묐뀈 �샊��� 留먮뀈�쓽 �돱�윴 �뙆愿댁쓽
�떆�옉泥섎읆 �룆�꽦 怨듦꼍�뿉 ����빆�븯�뒗 �뒫�젰�쓽 洹좏삎 �냼�떎
- 諛쒓껄�맂 �넀�긽�뱾
�몺 SALS, SOD1-related FALS�쓽 二쎌�� 議곗쭅�꽭�룷瑜� 遺꾩꽍�븳 寃곌낵
�넂 �떒諛깆쭏, 吏�吏�, DNA �궛�솕�쟻 �넀�긽 利앷�� 諛쒓껄
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Oxidative Stress
Oxidative stress;�궛�솕�쟻 �뒪�듃�젅�뒪
- 諛쒓껄�맂 �넀�긽�뱾
�몼 mouse model
- presymptomatic 吏덈퀝�떒怨꾩쓽 �슫�룞�돱�윴,
oligodendrocytes�뿉�꽌 mRNA �궛�솕
�넂 �떒諛깆쭏�쓽 諛쒗쁽�쓽 媛먯냼 諛쒓껄
�몾 TAR DNA-binding protein (TDP-43)-related ALS �룎�뿰蹂��씠�쓽
�꽭�룷 紐⑤뜽
- �슫�룞�돱�윴�꽭�룷 怨꾪넻�뿉�꽌 �궛�솕�쟻 �뒪�듃�젅�뒪媛� �쑀諛쒕릺�뒗 寃껋쓣 �솗�씤
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