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ᮏ❶䛷䛿䠈䝅䝱䝨䝻䝙䞁

GroEL

䜘䜚༢㞳䛧䛯䜰䝢䜹䝹䝗䝯䜲䞁(GroEL-AD) 䜢స〇䛧䠈A䃑

1-42䠈䃐-Synuclein䠈GroES

䛾䜰䝭䝻䜲䝗⥺⥔䛻ᑐ䛩䜛จ㞟ᢚไຠᯝ䜢᳨ウ䛧䛯䚹㐣๫㔞䛾

GroEL-AD

䜢ῧຍ䛧䛯᮲௳䛻䛚䛔䛶

A䃑1-42䠈䃐-Synuclein䠈GroES

䛾䜰䝭䝻䜲䝗⥺⥔ᙧ ᡂ䛿᏶඲䛻ᢚไ䛥䜜䜛䛾䜢♧䛧䛯(ᅗ

4-4)䚹䃐-Synuclein

GroES

䛾⥺⥔ᙧᡂ䛻䛚䛔䛶䛿䠈 㐣๫㔞䛾

GroEL-AD

䜢ῧຍ䛧䛺䛟䛶䜒䠈༑ศ䛺ᢚไຠᯝ䛜䜏䜙䜜䛯䚹䛧䛛䛧䠈

A䃑1-42

䛷䛿

᏶඲䛺ᢚไຠᯝ䜢ᚓ䜛䛯䜑䛻䛿

20

ಸ㔞௨ୖ䛾

GroEL-AD

䛜ᚲせ䛷䛒䛳䛯䚹䃐-Synuclein 䜔

GroES

䛷䛿᏶඲䛺ᢚไ䛜䜏䜙䜜䛯⃰ᗘ䛻䛚䛔䛶䜒䠈A䃑1-42 䛷䛿᏶඲䛺ᢚไ䜢♧䛥䛺䛛 䛳䛯(ᅗ

4-4d)䚹᏶඲䛻⥺⥔໬䜢ᢚไ䛩䜛㔞䜘䜚ప䛔⃰ᗘ䛾 GroEL-AD

䜢ῧຍ䛩䜛䛸䠈จ㞟 ഴྥ䛾䛒䜛䝍䞁䝟䜽㉁ศᏊ䛸┦஫స⏝䛩䜛䛣䛸䛷䠈᭱⤊ⓗ䛻ᙧᡂ䛩䜛⥺⥔㔞䛜ῶᑡ䛧䛶䛔䜛䛸

⪃䛘䜙䜜䛯䚹GroEL-AD 䛾Ꮡᅾୗ䛻䛚䛔䛶ᙧᡂ䛧䛯⥺⥔䛾ᙧែ䛻䛿㐪䛔䛜䜏䜙䜜䛺䛛䛳䛯䚹 䛣䜜䛿䠈ᚑ᮶⪃䛘䜙䜜䛶䛔䜛䜰䝭䝻䜲䝗⥺⥔䜢ᙧᡂ䛩䜛ᶵᵓ䛜཯ᫎ䛥䜜䛶䛔䜛䛸⪃䛘䜙䜜䜛(ᅗ

4-5䠈ᅗ 4-6)䚹䛧䛛䛧䠈䃐-Synuclein

䛾ሙྜ䛿౛እ䛷䠈GroEL-ADᏑᅾୗ䛻䛚䛔䛶ᙧᡂ䛧䛯

⥺⥔䛾

TEM

⏬ീ䛿䃐-Synuclein ༢⊂ཬ䜃⥺⥔ᙧᡂ䛻↓㛵ಀ䛺

BSA

䛾Ꮡᅾୗ䛻䛚䛔䛶 ᙧᡂ䛧䛯⥺⥔䜘䜚䜒⣽䛟䜏䜙䜜䛯(ᅗ

4-6)䚹ᅗ 4-9

QCM

ᐇ㦂䛻䛚䛡䜛

GroEL-AD 䛸䃐-Synuclein

㛫 䛾 ┦ ஫ స ⏝ ほ   䛥 䜜 䛯 」 ᩘ 䛾 ⤖ ྜ ᵝ ᘧ 䛾 䛯 䜑 䛻 䠈

GroEL-AD

䛜 䃐

-Synuclein

䛾⥺⥔ఙ㛗䜢ᢚไ䛧䛶䛚䜚䠈䛥䜙䛻ᙧᡂ䛩䜛⥺⥔䛾ᙧែ䜢ኚ໬䛩䜛⬟ຊ䜒᭷䛧䛶

䛔䜛䛣䛸䛜⪃䛘䜙䜜䛯䚹

ᮏ❶䛻䛚䛔䛶☜ㄆ䛥䜜䛯GroEL-AD䛾ᵝ䚻䛺ᶆⓗ䝍䞁䝟䜽㉁䛾⥺⥔ᙧᡂ䜢ᙉᅛ䛻ᢚไ䛩 䜛⬟ຊ䛿䠈ᵝ䚻䛺ᐇ㦂᮲௳ୗ䛻䛚䛔䛶䛥䜙䛻᫂䜙䛛䛸䛺䛳䛯䚹䜎䛪䠈GroEL-AD䛿䠈42䜰䝭䝜 㓟 ṧ ᇶ 䛾 ▷ 䛔A䃑

1-42

䝨 䝥䝏 䝗 䛸 䠈97䜰䝭 䝜 㓟 ṧ ᇶ 䛾

GroES

ཬ 䜃140䜰䝭 䝜 㓟 ṧ ᇶ 䛾

䃐-Synuclein

䛾ᬑ㏻䛾኱䛝䛥䛾୧᪉䜢ᶆⓗ䛸䛧䛶⤖ྜ䛷䛝䛯䚹䛭䜜䛻ຍ䛘䠈䛣䜜䜙ᶆⓗ䛸䛺䜛䝍 䞁 䝟 䜽 ㉁ 䛾 ᵓ 㐀 䛿 ᵝ 䚻 䛷 䠈 ▷ 䛔 䝫 䝸 䝨 䝥䝏 䝗䛷 䛒 䜛

A

䃑1-42䠈 ኳ ↛ ኚ ᛶ 䝍䞁 䝟 䜽 ㉁ 䛾 䃐

-Synuclein䠈䛭䛧䛶䝛䜲䝔䜱䝤ᵓ㐀䛾䜸䝸䝂䝬䞊䛜㒊ศⓗ䛻ኚᛶ䛧䛯 GroES䛜Ꮡᅾ䛩䜛䚹䛭䜜

121

䛻䜒䛛䛛䜟䜙䛪䠈ಶ䚻䛾䝍䞁䝟䜽㉁䛻䛚䛔䛶ほ   䛥䜜䜛䛭䜜䛮䜜䛾⥺⥔ ᙧ ᡂ ᮲ ௳䛻䛚䛔䛶

GroEL-AD䛿⤖ྜ䛻క䛔䜰䝭䝻䜲䝗⥺⥔จ㞟䜢ไᚚ䛩䜛䛣䛸䛜䛷䛝䛯䚹䛧䛛䛧䠈QCM䛻䜘䜛⤖

ྜ䛾ศᯒ䛻䛚䛔䛶䠈ᶆⓗ 䝍䞁䝟䜽㉁䛸GroEL-AD䛾⤖ྜ䛻䛚䛡䜛⤖ྜᵝᘧ䛿୍ᐃ䛷䛿䛺䛔 䛾䛜☜ㄆ䛥䜜䛯䚹䛣䜜䛿䠈3䛴䛾ᶆⓗ䝍䞁䝟䜽㉁䛸GroEL-AD䛸䛾⤖ྜᶵᵓ䛻䛚䛡䜛␗䛺䜛จ 㞟ᶵᵓ䛜཯ᫎ䛥䜜䛶䛔䜛䛸⪃䛘䜙䜜䜛䚹

GroEL䛿䠈⣽⬊ෆ䝇䝖䝺䝇䛻䜘䜚୍᫬ⓗ䛻⵳✚䛧䛯䜰䞁䝣䜷䞊䝹䝗䝍䞁䝟䜽㉁ศᏊ䜈䛾␯Ỉ

ⓗឤཷᛶ䛜䜘䛟▱䜙䜜䛶䛔䜛䚹GroEL䛾䜰䝢䜹䝹䝗䝯䜲䞁䛿䠈䛣䜜䜙䛾ศᏊ䜢༊ู䛧䠈⤖ྜ䛩䜛

␯Ỉᛶ䝉䞁䝃䞊䛸䛧䛶ാ䛟䝗䝯䜲䞁䛷䛒䜛䛾䛜❧ド䛥䜜䛶䛔䜛(ᅗ4-1䠈䝦䝸䝑䜽䝇H䠈I)䚹ᮏ❶䛷 䛿䛥䜙䛻䠈3䛴䛾䜰䝭䝻䜲䝗⥺⥔໬䛜⏕䛨䜛ᶆⓗ䝍䞁䝟䜽㉁䛻ᑐ䛩䜛␯Ỉᛶຠᯝ䜈䛾ᐤ୚䜢♧

䛧䛯䚹⥺⥔໬䝍䞁䝟䜽㉁䛻䛚䛡䜛␯Ỉᛶ䛾ᙺ๭䛿䠈BSA䜢䝁䞁䝖䝻䞊䝹䛸䛧䛯ᐇ㦂䛻䛚䛔䛶䜘䜚 ヲ⣽䛻☜ㄆ䛥䜜䛯(ᅗ4-6)䚹BSA䛿䠈ᶆⓗ䝍䞁䝟䜽㉁䛻ᑐ䛧䛶ᙳ㡪䛜䛺䛔⬡㉁䛸⤖ྜ䛧䠈⾑ὶ 䜢㏻ 䛧䛶ᵝ 䚻䛺ᰤ 㣴 䛸䛺䜛ศ Ꮚ 䜢㍺ ㏦ 䛩䜛⾑ Ύ 䝍䞁䝟䜽㉁ 䛷䛒䜛䚹BSA䛿䠈A䃑1-42

䠈䃐-Synuclein䠈GroES

䛾⥺ ⥔ ᙧ ᡂ 䛻䛚䛔䛶ᙳ 㡪 䜢ཬ 䜌䛧䛶䛔䜛䜘䛖䛻䜏䜙䜜䜛䛜䠈➼ ⃰ ᗘ 䛾

GroEL-AD䜢ῧຍ䛧䛯᮲௳䛸ẚ㍑䛧䛶䛭䛾ᙳ㡪䛿ᙅ䛔(ᅗ4-6)䚹䛣䛾䝁䞁䝖䝻䞊䝹ᐇ㦂䛻䛚䛔

䛶䠈GroES䛷䛿᰾ᙧᡂ䛻䠈A䃑1-42䛷䛿ᙧᡂ䛥䜜䜛⥺⥔䛾ᙧែ䛜ⴭ䛧䛟ኚ໬䛧䛶䛔䜛䜘䛖䛻䠈

BSA䛿䛭䜜䛮䜜䛾ᶆⓗ䝍䞁䝟䜽㉁䛾⥺⥔ᙧᡂ䛻ᑐ䛧␗䛺䜛ᙳ㡪䜢ཬ䜌䛧䛶䛔䜛䚹䛣䛾⤖ᯝ䜘

䜚䠈䜰䝭䝻䜲䝗⥺⥔ᙧᡂ㐣⛬䛾᰾ᙧᡂ䛸⥺⥔ఙ㛗䛻䛚䛡䜛␯Ỉᛶ┦஫స⏝䛿ከ✀ከᵝ䛺ᵝᘧ 䜢䜒䛳䛶䛔䜛䛣䛸䛜♧䛥䜜䛯䚹䛭䜜䛻䠈A䃑1-40䛾⥺ ⥔ᙧ ᡂ䛻㛵䛩䜛௨ ๓ 䛾◊✲䛻䛚䛔䛶䠈 ᵝ䚻䛺ṇ䛸㈇䛾㟁Ⲵ䜢䜒䛴໬ྜ≀䜢⏝䛔䛯⥺⥔໬䛻䛚䛡䜛㟼㟁┦஫స⏝䛜㛵୚䛧䛶䛔䜛䛣䛸 䜒ሗ ࿌ 䛥䜜䛶䛚䜚䠈␯ Ỉ ᛶ ┦ ஫ స ⏝ 䛰䛡䛜⥺ ⥔ ໬ 䜢ㄪ ᩚ 䛧䛶䛔䜛䛾䛷䛿䛺䛔䛸⪃ 䛘䜙䜜䜛

(115,116)䚹እᅉ䛻䜘䜛่⃭䜈䛾ឤཷᛶ䛻ᑐ䛩䜛䝍䞁䝟䜽㉁䛾⥺⥔໬䛻䛿ከ䛟䛾┦஫స⏝䛜

㛵୚䛧䛶䛔䜛䚹䛣䜜䛿䠈䝍䞁䝟䜽㉁䛸䝍䞁䝟䜽㉁䛾┦஫స⏝䛻䜘䜛⥺⥔໬䛾NMR䜔㉁㔞ศᯒ 䛺䛹䛾ཎᏊ䝺䝧䝹䛻䛚䛡䜛ศᯒ䜘䜚᫂䜙䛛䛸䛺䜛䛷䛒䜝䛖䚹

122

➨ 5 ❶㻌 ⥲ྜ⪃ᐹ

ᮏ◊✲䛷䛿䠈➨

2

❶䛷

GroEL G192X

ኚ␗య䛻䜘䜛䝅䝱䝨䝻䝙䞁ᶵ⬟ゎᯒ䠈➨

3

❶䛷

GroEL G192X

䜢⏝䛔䛯ኳ↛ኚᛶ䝍䞁䝟䜽㉁䛾┦஫స⏝䛸䜰䝭䝻䜲䝗⥺⥔ᙧᡂ䜈䛾㛵୚䛻䛴

䛔䛶䠈➨

4

❶䛷䛿

GroEL

䛾䜰䝢䜹䝹䝗䝯䜲䞁䜢༢㞳䛧䛯

GroEL-AD

䜢⏝䛔䛶䠈䜰䝭䝻䜲䝗⥺⥔

ᙧᡂ䜈䛾㛵୚䜢ホ౯䛧䛯䚹ᮏ❶䛷䛿௨ୖ䛾⤖ᯝ䜢䜅䜎䛘䛶䠈GroEL 䛻䛚䛡䜛䜰䝢䜹䝹䝗䝯䜲 䞁䛜㛵୚䛧䛶䛔䜛䜰䝭䝻䜲䝗⥺⥔ᙧᡂ䜈䛾㔜せ䛺ᙺ๭䛻䛴䛔䛶⥲ྜ⪃ᐹ䛩䜛䚹

2

❶䛻䛚䛔䛶

GroEL WT

Hinge䊡㒊఩䛻䛚䛡䜛 Gly192

ṧᇶ䜢䝣䜯䞁䝕䝹䝽䞊䝹䝇 య✚䛻ὀ┠䛧䛶ᵝ䚻䛺䜰䝭䝜㓟ṧᇶ䛻⨨᥮䛧䛯

GroEL G192X

ኚ␗య䛿䠈኱䛝䛔䜰䝭䝜㓟ṧ ᇶ䛻⨨᥮䛧䛯ኚ␗య䜋䛹䝅䝱䝨䝻䝙䞁ᶵ⬟䛻ᙳ㡪䜢ཬ䜌䛧䛶䛔䜛䛣䛸䜢♧䛧䛯䚹䛭䛣䛷䠈5-1 䛻 䛶

GroEL

Hinge

䊡㒊఩䛻䛚䛡䜛

Gly192

ṧᇶ䛾ᙺ๭䛻䛴䛔䛶㏙䜉䜛䚹

䛥䜙䛻䠈➨

3

❶䜘䜚

GroEL G192X

ኚ␗య䛿ኚ␗య㛫䛻䛚䛔䛶⥺⥔ᙧᡂ䛾␗䛺䜛㐣⛬䛻 ᙳ㡪䜢ཬ䜌䛧䠈ᙧᡂ䛥䜜䛯⥺⥔䛾ᙧែ䜒ኚ໬䛧䛶䛔䛯䚹䛭䛣䛷䠈5-2 䛻䛶

GroEL

Gly192

ṧᇶ䛾ኚ໬䛧䛯䛣䛸䛻䜘䜛⥺⥔ᙧᡂ䜈䛾ᙳ㡪䛻䛴䛔䛶⪃ᐹ䛩䜛䚹

䜎䛯䠈➨

4

❶䜘䜚䠈GroEL-AD䛜䜰䝭䝻䜲䝗⥺⥔ᙧᡂ䛾ᢚไ䜢⾜䛳䛶䛔䜛䛾䜢♧䛧䛯䚹䛭䛣䛷䠈

5-3

䛻䛶䜰䝭䝻䜲䝗⥺⥔䜢ᙧᡂ䛩䜛䝍䞁䝟䜽㉁䛻ᑐ䛩䜛

GroEL-AD

䛾ᐤ୚䛻䛴䛔䛶㆟ㄽ䛩䜛䚹

5-1䠊Hinge䊡㒊఩䛻Ꮡᅾ䛩䜛 Gly192 ṧᇶ䛾 GroEL 䛻䛚䛡䜛ᙺ๭

GroEL

Hinge䊡㒊఩䛻䛚䛡䜛 Gly192

ṧᇶ䛿䠈䜰䝭䝜㓟ṧᇶ䜢ኚ໬䛥䛫䛶䜒䛭䛾஧ḟ ᵓ㐀䛸ᅄḟᵓ㐀䛻኱䛝䛺㐪䛔䛿䛺䛛䛳䛯(ᅗ

2-5䠈2-7)䚹୍᪉䛷䠈ANS

⺯ග ᐃ䛻䜘䜛⾲㠃␯

Ỉᛶ䛾ゎᯒ䛛䜙䠈Gly192ṧᇶ䛾⨨᥮䛧䛯䜰䝭䝜㓟ṧᇶ䛜኱䛝䛔䜋䛹⾲㠃␯Ỉᛶ䛜㧗䛟䛺䛳䛶 䛔䛯(ᅗ

2-4)䚹GroEL

䛾ෆ㒊䛿䠈␯Ỉᛶ㡿ᇦ䛜ከ䛔䛸䛥䜜䛶䛔䜛(16)䚹Gly192ṧᇶ䜢ኚ໬䛥 䛫䛯䛣䛸䛷

GroEL

Open

ᵓ㐀䛻䛺䜚䠈ANS 䛻䜘䜛

GroEL

䛾䜰䝢䜹䝹䝗䝯䜲䞁䜈䛾⤖ྜ䛜 ኚ ໬ 䛩 䜛 䛣 䛸 䛷 䠈

ANS

⺯ ග ᙉ ᗘ 䛾 ኚ ໬ 䛻 ᙳ 㡪 䛧 䛶 䛔 䜛 䛣 䛸 䛜 ⪃ 䛘 䜙 䜜 䛯 䚹

Pyrococcus

123

furiosus

⏤᮶䛾䜾䝹䞊䝥䊡ᆺ䝅䝱䝨䝻䝙䞁(PfCPN)䛻䛚䛔䛶䠈Gly345 ṧᇶ䜢

Asp

䛻⨨᥮

䛧䛯

G345D

ኚ␗య䛿䠈Co(2+)䛾Ꮡᅾୗ䛻䛚䛔䛶㔝⏕ᆺ䛾⣙

25%䛧䛛 ATP/ADP

ຍỈศ ゎάᛶ䜢♧䛥䛪䠈

in vitro

䛻䛚䛔䛶ኚᛶ

MDH

䛾䝣䜷䞊䝹䝕䜱䞁䜾௓ຓ䛾⬟ຊ䛜ῶᑡ䛧䛶䛔 䛯(117)䚹Gly-345 䛿ྂ⣽⳦䜾䝹䞊䝥䊡ᆺ䝅䝱䝨䝻䝙䞁䛻㧗䛟ಖᏑ䛥䜜䛶䛚䜚䠈䜰䝢䜹䝹䝗䝯䜲 䞁䛸䜲䞁䝍䞊䝯䝕䜱䜶䜲䝖䝗䝯䜲䞁䛾㛫௜㏆䛻Ꮡᅾ䛧䛶䛔䜛䚹䝅䝱䝨䝻䝙䞁䛿ኚᛶᇶ㉁䛾䝸䝣䜷䞊 䝹䝕䜱䞁䜾䜢⿵ຓ䛩䜛㝿䛻኱つᶍ䛺ᵓ㐀ኚ໬䛜⏕䛨䜛䛣䛸䛜▱䜙䜜䛶䛔䜛䚹䛭䛾䛯䜑䠈䝅䝱䝨 䝻䝙䞁䛜ᶵ⬟䜢ᯝ䛯䛩䛻䛒䛯䜚䠈ᵓ㐀ኚ໬䛾ᨭⅬ䛸䛺䜛㒊఩䛻Ꮡᅾ䛩䜛䜰䝭䝜㓟䜢⨨᥮䛩䜛䛣 䛸䛷䠈ᵓ㐀ኚ໬䛸䛭䜜䛻క䛖ᶵ⬟Ⓨ⌧䛻䛚䛡䜛ᙺ๭䜢ヲ⣽䛻ㄪ䜉䜛䛣䛸䛜䛷䛝䜛䚹GroEL 䛻 䛚䛡䜛

Gly192

ṧᇶ䜢ኚ໬䛥䛫䛯䛣䛸䛻䜘䜛䝅䝱䝨䝻䝙䞁ᶵ⬟䜈䛾ᙳ㡪䜢ㄪ䜉䛯䛸䛣䜝䠈ATP 䛾ຍỈศゎ⬟ຊ䠈MDH 䛸

Rhodanese

䜢ᇶ㉁䛸䛧䛶⏝䛔䛯䝸䝣䜷䞊䝹䝕䜱䞁䜾௓ຓ⬟ຊ䛿䠈

Gly192

ṧᇶ䜢⨨᥮䛧䛯䜰䝭䝜㓟ṧᇶ䛜኱䛝䛔䜋䛹䛭䜜䛮䜜䛾άᛶ䛜పୗ䛧䛶䛔䛯䚹Zachary

W䠊White

䜙䛿䠈GroEL 䛻䛚䛡䜛

3

␒┠䛾

Lys

Glu

ṧᇶ䛻⨨᥮䛧䛯

GroEL K3E

ኚ␗

య䜢⏝䛔䛯◊✲䛻䛚䛔䛶䠈GroEL K3E 䛿䠈༢㔞యᵓ㐀䛷䛒䜛䛜䠈䛭䛾஧ḟᵓ㐀䛿

GroEL WT

䛸㢮ఝ䛧䛶䛚䜚䠈GroEL WT䛸ẚ㍑䛧䠈㧗䛔

ANS

⺯ග䛜ほ 䛥䜜䛯䚹䛧䛛䛧䠈䝚䜽䝺䜸䝏 䝗Ꮡᅾୗ䛻䛚䛔䛶ኚᛶᇶ㉁䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾䜢⿵ຓ䛩䜛䛣䛸䛜䛷䛝䛺䛛䛳䛯(118)䚹௨๓䠈

Gly192

ṧᇶ䜢᭱䜒኱䛝䛔䜰䝭䝜㓟ṧᇶ䛷䛒䜛

Trp

䛻⨨᥮䛧䛯

GroEL G192W

ኚ␗య䛿䠈

ATP

䛾㠀Ꮡᅾୗ䛻䛚䛔䛶

cis䉄 ring

ഃ䛻

GroES

䛜⤖ྜ䛩䜛䛣䛸䜢ሗ࿌䛧䛯(19)䚹GroEL

G192X

ኚ␗య䛾䝅䝱䝨䝻䝙䞁ᶵ⬟ゎᯒ䛾⤖ᯝ䜘䜚䠈GroEL G192Xኚ␗య䛿䠈Gly192ṧᇶ 䜢⨨᥮䛧䛯䜰䝭䝜㓟䛻䜘䜚

GroES

䛸䛾┦஫స⏝䛻ኚ໬䛜⏕䛨䛶䛔䜛䛣䛸䛜⪃䛘䜙䜜䛯䚹QCM ἲ䛻䜘䜛

ATP

㠀Ꮡᅾୗ䛻䛚䛡䜛

GroEL G192X

ኚ␗య䛸

GroES

䛾┦஫స⏝䜢ゎᯒ䛧䛯⤖

ᯝ䛷䛿䠈Gly192 䜢኱䛝䛔䜰䝭䝜㓟ṧᇶ䛻⨨᥮䛧䛯ኚ␗య䜋䛹

GroES

䛸䛾┦஫స⏝䛜ᙉ䛔䛣 䛸䛜᫂䜙䛛䛸䛺䛳䛯(ᅗ

2-11)䚹

GroEL G192X

ኚ␗య䛻䛚䛡䜛

cis

trans

䉄ring䛾ാ䛝䜢䜘䜚ヲ⣽䛻ゎᯒ䛩䜛䛣䛸䜢┠ⓗ䛸 䛧䠈

cis

䉄ring䛾ᶵ⬟䛰䛡䜢䜒䛴

GroEL

䛾䝅䞁䜾䝹䝸䞁䜾ኚ␗య

SR-1

Gly192

䛻䛚䛡䜛䜰䝭

124

䝜㓟ṧᇶ䜢⨨᥮䛧䛯

SR-1 G192X

ኚ␗య䜢⏝䛔䛯䝅䝱䝨䝻䝙䞁ᶵ⬟ゎᯒ䜢⾜䛳䛯䚹MDH 䛾 䝸䝣䜷䞊䝹䝕䜱䞁䜾άᛶ ᐃ䛾⤖ᯝ䛻䛚䛔䛶䠈䝅䞁䜾䝹䝍䞊䞁䜸䞊䝞䞊(94)䛜ཎᅉ䛷άᛶ䜢 䜋䛸䜣䛹♧䛥䛺䛔

SR-1 WT

䛸ẚ㍑䛧䛶

SR-1 G192V

SR-1 G192I

ኚ␗య䛷䛿㧗䛔άᛶ䜢

♧䛧䛶䛔䛯䚹௨๓䛾◊✲䛻䛚䛔䛶䠈䝍䞁䝕䝮䛻⧅䛜䛳䛯

GroES 7

㔞య䛻䛚䛡䜛䛔䛟䛴䛛䛾

GroES

䝃䝤䝴䝙䝑䝖䜢

Asp

ṧᇶ䛻⨨᥮䛧䛯䛣䛸䛷䠈

SR-1

䛸䛾┦஫స⏝䛿ᙅ䜎䜛䛜䠈ኚᛶ

MDH

䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾άᛶ䛜

GroES WT

䜘䜚䜒ቑຍ䛧䛶䛔䜛䛣䛸䛜ሗ࿌䛥䜜䛶䛔䜛(119)䚹 䛥䜙䛻䠈GroEL 䛸┦஫స⏝䛩䜛䛣䛸䛜䛷䛝䜛䝃䝤䝴䝙䝑䝖䛾ᩘ䜢ኚ໬䛥䛫䛯

GroES

䛻䛚䛔䛶䠈

7

ဨ⎔

GroES

䝃䝤䝴䝙䝑䝖ᙜ䛯䜚

GroEL

䛸┦஫స⏝䛩䜛䛣䛸䛜䛷䛝䜛䝃䝤䝴䝙䝑䝖䛜

5

䛴Ꮡ ᅾ䛧䛶䛔䜛䛸

GroEL WT

GroES WT

䛸ྠ⛬ᗘ䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾άᛶ䜢♧䛩䛣䛸䛜ሗ࿌

䛥䜜䛶䛔䜛(120)䚹䛣䛾ሗ࿌䛷䛿䛥䜙䛻

GroES

䛾䝃䝤䝴䝙䝑䝖䛜㞄᥋䛧䛶䛔䜛䜋䛹㔝⏕ᆺ䛻㢮 ఝ䛧䛯ᶵ⬟䜢ಖᣢ䛩䜛䛣䛸䛜♧䛥䜜䛶䛔䜛䚹䛣䜜䜙䛾ሗ࿌䛛䜙䠈ᇶ㉁䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾䜢௓

ຓ䛩䜛䛻䛒䛯䜚䠈GroEL䛸

GroES

7

ဨ⎔ྠኈ䛜඲䛶⤖ྜ䛧䛶䛔䛺䛔ྍ⬟ᛶ䜒⪃䛘䜙䜜䜛䚹

SR-1 G192X

ኚ␗య䛾䝅䝱䝨䝻䝙䞁ᶵ⬟ゎᯒ䛾⤖ᯝ䜘䜚䠈GroEL G192Xኚ␗య䛾

cis

䉄ring 䛻䛚䛡䜛

GroES

䛸䛾┦஫స⏝䛿

Gly192

ṧᇶ䜢᭱䜒኱䛝䛔

Trp

䛻⨨᥮䛩䜛䛣䛸䛷᏶඲䛻

GroES

䛸⤖ྜ䛩䜛䛜䠈䛭䜜䜘䜚ᑠ䛥䛔䜰䝭䝜㓟䛷䛿᏶඲䛻䛿

GroES

䛸⤖ྜ䛧䛶䛔䛺䛔䛣䛸䛜⪃

䛘䜙䜜䜛䚹䛣䜜䜘䜚䠈GroEL G192Xኚ␗య䛻䛚䛡䜛

GroES

䛸䛾┦஫స⏝䛿䠈Gly192ṧᇶ䜢

⨨᥮䛩䜛䜰䝭䝜㓟ṧᇶ䜢㑅ᢥ䛩䜛䛣䛸䛷

Open

≧ែ䛾䜰䝢䜹䝹䝗䝯䜲䞁䜢ㄪ⠇䛩䜛䛣䛸䛷ኚ໬䛥 䛫䜛䛣䛸䛜ྍ⬟䛻䛺䜛䛸⪃䛘䜙䜜䜛䚹䛥䜙䛻䠈GroES䛸䛾┦஫స⏝䜢ኚ໬䛥䛫䜛䛣䛸䛷ኚᛶᇶ㉁

䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾௓ຓ⬟ຊ䜒ㄪ⠇䛩䜛䛣䛸䛜ྍ⬟䛸䛺䜛䛸⪃䛘䜙䜜䛯䚹

௨ୖ䜘䜚䠈Gly192 ṧᇶ䛿䠈GroEL 䛜ᮏ᮶᭷䛧䛶䛔䜛ኚᛶ䝍䞁䝟䜽㉁䛾䝸䝣䜷䞊䝹䝕䜱䞁䜾

⿵ຓ⬟ຊ䜢Ỵᐃ䛩䜛㔜せ䛺ᅉᏊ䛷䛒䜚䠈䛣䛾

Hinge䊡㒊఩䛻఩⨨䛩䜛ṧᇶ䜢ኚ໬䛥䛫䜛䛣䛸

GroEL

䛾䝅䝱䝨䝻䝙䞁ᶵ⬟䜢䝍䞁䝟䜽㉁ᕤᏛⓗ䛻ㄪᩚ䛩䜛䛣䛸䛜ྍ⬟䛷䛒䜛䛣䛸䛜♧၀䛥

䜜䛯䚹䛣䛾䛸䛝䛾⪃䛘䜙䜜䜛䝅䝱䝨䝻䝙䞁䝃䜲䜽䝹䜢ᅗ

5-1

䛻♧䛩䚹

125

5-1. GroEL WT GroEL G192Xኚ␗య䛾䝅䝱䝨䝻䝙䞁䝃䜲䜽䝹䛾ẚ㍑

ୖᅗ䠖GroEL G192Xኚ␗య䛾䝅䝱䝨䝻䝙䞁䝃䜲䜽䝹

Hinge䊡㒊఩䛾Gly192ṧᇶ䜢⨨᥮䛩䜛䛣䛸䛷䜰䝢䜹䝹䝗䝯䜲䞁䛾ഴ䛝䜢ኚ໬䛥䛫䛶䛔䜛(䕔ෆ)䚹 䛣䛾ഴ䛝䛾ኚ໬䛿Gly192䜢䝣䜯䞁䝕䝹䝽䞊䝹䝇య✚䛻䜘䜚⨨᥮䛧䛯ṧᇶ䛻䜘䜚ኚ໬䛩䜛䚹

GroEL G192X ኚ␗య䛿䠈䜰䝢䜹䝹䛜ୖ䛜䛳䛶䛔䜛 cis䉄ring 䛻䛿 ATP㠀Ꮡᅾୗ䛷GroES䛜⤖ྜ

䛩䜛䚹trans䉄ring䛻䛿ኚᛶᇶ㉁䛜⤖ྜ䛩䜛䚹䛭䛾ᚋ䠈ATP䛸䛥䜙䛺䜛GroES䛾ῧຍ䛷trans䉄ring ኚᛶᇶ㉁䛸 GroES 䛜⨨䛝᥮䜟䜛䚹⨨᥮䛧䛯䜰䝭䝜㓟ṧᇶ䛻䜘䛳䛶䛿୍㒊䛜䜻䝱䝡䝔䜱ෆ䛻ධ䜚㎸䜣䛷䝸 䝣䜷䞊䝹䝕䜱䞁䜾䛾௓ຓ䛜⾜䜟䜜䜛䚹

ୗᅗ䠖GroEL WT 䛾䝅䝱䝨䝻䝙䞁䝃䜲䜽䝹

GroEL WT 䛿ኚᛶᇶ㉁䜢cis䉄ring䛻⤖ྜ䛥䛫䛯ᚋ ATP䛾Ꮡᅾୗ䛷 GroES䛜⤖ྜ䛩䜛䛣䛸䛷䠈኱

つᶍ䛺ᵓ㐀ኚ໬䜢ᘬ䛝㉳䛣䛧ෆ㒊䛻䝉䞁䝖䝷䝹䜻䝱䝡䝔䜱䛸࿧䜀䜜䜛✵Ὕ䜢స䜚ฟ䛧䠈ኚᛶᇶ㉁䜢ⴠ䛸䛧

㎸䜐䚹䛭䛾ᚋኚᛶᇶ㉁䛜Ꮡᅾ䛧䛶䛔䜛≧ែ䛷䛿ኚᛶᇶ㉁䜢 trans䉄ring ഃ䛻⤖ྜ䛩䜛䚹⤖ྜ䛧䛶䛔䜛 ATPADP䛻ຍỈศゎ䛥䜜䜛䛸GroES䛜እ䜜䠈ኚᛶᇶ㉁䛜እ䛻ᨺฟ䛥䜜䜛䚹

126

55-2䠊ኳ↛ኚᛶ䝍䞁䝟䜽㉁䛾䜰䝭䝻䜲䝗⥺⥔ᙧᡂ㐣⛬䛻䛚䛡䜛 GroEL

䛾 Hinge䊡㒊఩䛻Ꮡᅾ䛩䜛 Gly192 ṧᇶ䛾ᙺ๭

GroEL

Hinge䊡㒊఩䛿䜰䝢䜹䝹䝗䝯䜲䞁䛾ືస䛻㔜せ䛺ᙺ๭䜢ᯝ䛯䛧䠈䛣䛾㒊఩䛻Ꮡᅾ

䛧䛶䛔䜛

Gly192

ṧᇶ䜢௚䛾䜰䝭䝜㓟ṧᇶ䛻⨨᥮䛩䜛䛣䛸䛷䜰䝢䜹䝹䝗䝯䜲䞁䛾ື䛝䜢ㄪᩚ䛷䛝 䜛ྍ⬟ᛶ䛿๓⠇䛷♧䛧䛯䚹GroEL䛾䜰䝢䜹䝹䝗䝯䜲䞁䛷䛿䠈ᇶ㉁䛾⤖ྜ㒊఩䛸

GroES

䛾⤖ྜ

㒊 ఩ 䛿㢮 ఝ 䛧䛯఩ ⨨ 䛻Ꮡ ᅾ 䛧䛶䛔䜛

(121)䚹Gly192

䜢௚ 䛾䜰䝭䝜㓟 ṧ ᇶ 䛻⨨ ᥮ 䛧䛯䛣䛸䛷

Open

≧ែ䛻䛺䜛䛸

GroEL G192X

ኚ␗య䛿

ATP

䛾㠀Ꮡᅾୗ䛷

GroES

䛜⤖ྜ䛩䜛䚹䛣䜜 䛻䜘䜚ኚᛶ≧ែ䛾

MDH

Rhodanese

䛿䝉䞁䝖䝷䝹䜻䝱䝡䝔䜱ෆ䛻ධ䜛䛣䛸䛜䛷䛝䛪䠈䝸䝣䜷䞊 䝹䝕䜱䞁䜾䜢௓ຓ䛷䛝䛺䛔䛣䛸䛜♧䛥䜜䛯䚹ANS⺯ග ᐃ䛾⤖ᯝ䛻䛚䛔䛶䠈Gly192ṧᇶ䜢⨨

᥮ 䛧䛯 䜰䝭䝜 㓟 ṧ ᇶ 䛜 ኱ 䛝䛔䜋䛹

Open

≧ ែ 䛸 䛺䜚䜰䝢䜹䝹䝗䝯䜲䞁䛜 ⁐ ᾮ ୰ 䛻 ᭚ 䛥䜜䛯

G192X

ኚ␗య䜋䛹⾲㠃␯Ỉᛶ䛜㧗䛟䛺䛳䛯䛣䛸䛛䜙䠈䛭䛾⾲㠃䛻␯Ỉᛶṧᇶ䜢ከ䛟ྵ䜣䛷䛔

䜛ኳ↛ኚᛶ䝍䞁䝟䜽㉁䛸䜘䜚┦஫స⏝䛧䜔䛩䛔䛣䛸䛜⪃䛘䜙䜜䛯䚹⮅⮚䛛䜙ศἪ䛥䜜䜛䝨䝥䝏䝗 䝩䝹䝰䞁䛾୍䛴䛷䛒䜛䜲䞁䝇䝸䞁䛿䠈䜰䝭䝻䜲䝗⥺⥔◊✲䛾䝰䝕䝹䝍䞁䝟䜽㉁䛸䛧䛶䛥䜜䛶䛔䜛

(122)䚹䛣䜜䜎䛷䠈䜾䝹䞊䝥䊡ᆺ䝅䝱䝨䝻䝙䞁䛻ᒓ䛩䜛㉸㧗⇕ᛶ䛾ྂ⣽⳦⏤᮶䛾䝅䝱䝨䝻䝙䞁

(HSP60)䛾 C

ᮎ➃䛻䛚䛡䜛ᰂ㌾ᛶ䛜ቑຍ䛧䠈㧗 ᮲௳ୗ䛷䛺䛟䛸䜒㧗䛔άᛶ䜢♧䛩ኚ␗య

䛻䛚䛔䛶䠈䜲䞁䝇䝸䞁䛜ᙧ ᡂ 䛩䜛⥺ ⥔ ᩿ ∦ 䛸⤖ ྜ 䛧䠈⥺ ⥔ 䜢ศ ゎ 䛩䜛䛣䛸䛜 ሗ ࿌ 䛥䜜䛶䛔䜛

(123)䚹䛣䛾 HSP60

ATP

䛾Ꮡᅾୗ䛻䛚䛔䛶䜲䞁䝇䝸䞁⥺⥔䛾

Thioflavin-T

⺯ග䛜

ATP

㠀Ꮡᅾୗ䜘䜚䜒పୗ䛧䛶䛚䜚䠈ATPᏑᅾୗ䛷䃑䝅䞊䝖䛜䛥䜙䛻ῶᑡ䛧䛶䛔䜛䛾䛜♧䛥䜜䛶䛔䜛䚹 䛥䜙䛻䠈SHSY5Y ⣽⬊䜢౑⏝䛧䛯

MTT

ศᯒ䛻䛚䛔䛶䠈 HSP60 䛸

ATP

Ꮡᅾୗ䛻䛚䛔䛶䜰 䝭䝻䜲䝗⥺⥔䛿⬺จ㞟䛥䜜䠈⥺⥔䛾䜏䛸ẚ㍑䛧䛶≉䛻㧗䛔ẘᛶ⏘≀䜢ᙧᡂ䛧䛯䚹ATP 㠀Ꮡᅾ

ୗ䛻䛚䛡䜛

HSP60

䛿䜲䞁䝇䝸䞁⥺⥔䛾䜏䛸ྠ⛬ᗘ䛾ẘᛶ䛷䛒䛳䛯䚹ATP Ꮡᅾୗ䛷

HSP60

䛿⥺⥔䛾⬺ᵓ⠏䜢

ATP

㠀Ꮡᅾୗ䜘䜚䜒ຍ㏿䛩䜛䛣䛸䛷䠈⥺⥔䜘䜚ẘᛶ䛜㧗䛔䜰䝰䝹䝣䜯䝇䛺 จ㞟䛸⥺⥔᩿∦䜢⏕⏘䛩䜛䛣䛸䛷ẘᛶ䛜ቑຍ䛧䛶䛔䜛䛣䛸䛜⪃䛘䜙䜜䛶䛔䜛(124)䚹䛣䜜䜘䜚䠈