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1 136ム ァ ァ9 2009; 21 (4): ゙ fiapple ヲ apple apple ケ0 (09 閨03080ケ0 I: ァ8 ゙ ァ ク7 apple ク ク7) メ. メ. メ080ク ヲ ケ apple086ヲ003080ク ァ ヲ apple ヲ ァ apple ヲ ク ァ80ケ apple086ァ ケ ク700 閨 ァ ァ ァ8, 02 08apple086ァ9 apple ヲ fi 040ク fi 02080ク7 04apple ヲ ヲ ヲ0. メ ケ ク702 6ァ9 0ケ ァ9 0ケ apple ヲ 閨 apple 閨 ァ ァ80ケ0 閨03000ク707 0ケ0, applefi apple086ァ9 閨 apple ヲ fi 閨 fi 03fi apple 閨 ケ0 04apple ァ80ケ ケ0. 6セ40208 apple 閨03080ケ ケ0 apple 03086ヲ004 0ケ fiapple ケ fi 閨 ケ ァ9 0ケ ァ ク703096ァ9 020キ307 apple ク キ307. 閨 ケ : 02026ァ90709, ァ90709, apple ヲ , 閨0ケ E キ3096ァ8 ヲ fi 04apple006ァ903 00, apple08036ヲ0apple ク700 閨 ァ ク7, 040ク fi fi fi 02080ク ヲ0, apple ケ ァ apple086ァ ァ apple ク ァ apple03fi080ク70308, apple080ク ク707 閨 閨 ク7 Bowman. O 04apple fi0ケ fi0ケ fi0ケ0, apple0602fi 00apple6ァ9apple00 08, apple ァ applefi ヲ ク ク キ ヲ ァ9080ク7, apple080ク7 apple ヲ キ ァ9040キ キ307 04apple , apple ァ ァ (6セ4ヲフ09) apple ヲ , apple080ク7 00apple0007 6ヲ0080ク 閨010キ3 00apple ケ0. K0ク fi0ケ0 026ヲ ケ ァ ク7 apple ク ク fi ヲ 006ァ800, 縺 fi ァ908 ゙ ヲミappleapple ゙ fi 6ム ァ , ヲミ ァ8 6セ ァ8, 6ム fi メ apple fi apple ァ ゙ ヲ apple ヲ apple ヲ0apple02080ク 閨 applefi ク ヲ ヲ ク ク ヲ apple , apple ケ ケ0 apple03fi ケ 閨0ケ0 applefi 02060ケ0 08apple086ァ9000ケ0 03 apple ヲ ク apple ヲ O 020ク fi0ケ0 026ヲ ケ0 020キ apple 閨070キ307 apple ク キ ァ apple 6ヲ ゙0ク702fi 006ァ apple0802 閨 ケ apple ケ0 閨 ケ0 020キ ァ90キ307 apple080ク7 apple ク apple03fi ク7 020ク ヲ0 026ヲ ク7, fiapple0キ30ケ ク702036ァ907000ケ0 (cyclins) ケ0 applefi 02060ケ0 020ク702036ァ907000ケ ケ0 (cyclin-dependent kinases, CDK), キ

2 13 メ. メO08ヲ 06O08ヲ 6セ4O6セ4,. 03 ゙ メO09O08 O08 6ム ァ o03o096ァ9 21, ァ ケ キ302000ケ107000ケ0 020キ307 apple apple セ40ク ァ ケ0: 6ム8 メ: 0409: ァ8 apple030キ302000ケ10709, 6ム ク apple030キ302000ケ10709, 6セ404: 閨0ケ キ ク70ケ0, 020キ 閨0キ ケ ケ0 020キ ク (cyclin kinase inhibitors). 020ク , fiapple0キ30ケ0 閨 ァ ァ9000ケ ヲ ァ ク ァ9 0ケ0, apple ヲ ィB080ク707 apple030キ302000ケ107000ケ0 apple080ク 閨 ク ク fi 026ヲ , fiapple0キ30ケ p27, p21, p57, apple 03080ク ィB080ク キ300 閨0709 閨 apple030キ302000ク apple080ク apple ク707, fiapple0キ30ケ ク702036ァ907000ケ0 D, A, B Ki ヲ apple ヲ ァ9ィB applefi 006ァ ク700 閨 ァ ァ ァ8 4. ゙apple ヲ applefi 閨07 000ク 閨03000ケ0 020ク fi , applefi apple086ァ ヲ007 apple030キ ァ90ケ ァ ケ0 apple080ク ァ apple03080ケ apple ァ ァ809080ク apple08-030ク ケ0 000ク ァ90ケ0 apple 閨0ケ0, 02060ケ0 apple ァ90ケ0 apple 閨0ケ0 (0909), 00 閨040キ3 020キ307 08apple086ァ90キ307 apple セ4ヲフ09 (6ム ). O apple ヲ ク7036ヲ 閨0708, fi ク fi ク7, fi ァ ク7 apple ク ク7. 09 閨040キ3 020キ apple ヲ ク707 閨 セ4ヲフ09, apple ヲ O apple ァ9ィB ケ0 閨07 apple08036ヲ0apple ァ902020ク708 apple080ク7 apple ッ ァ803080ク apple ァ8 (6ム ). ヲ ァ ク70ケ ァ9ィB ク applefi 閨 ク700 閨0708 6ァ902020ク ァ90キ ァ907090ケ0, apple086ァ908 apple ァ ァ ク7 020ク ヲ0. ヲ ヲ0 020キ apple ク キ307, apple ク70ケ , ク applefi 006ァ9 apple ァ800 apple080ク E ヌ apple08036ヲ0apple ァ ク7 apple ク ク7 fiapple0キ30ケ0 apple ァ9ィb ァ ヲ apple6ァ9080ク fi ケ0 閨 ケ0 ( ) キ304090ケ0 ( ). 0909: ァ90ケ0 apple 閨0ケ0 6セ404: 6セ 閨0ケ ( ァ8 applefi 0208 Histology Learning System Boston University / ク7 02fi0ケ0 02fiapple080ケ0: histology). 274

3 136ム ァ ァ9 21, 2009 E O ケ 閨0ケ ァ ァ8 046ヲ apple 閨0ケ0 020キ307 apple apple ( ヌ fi fiapple fi ケ0 閨 ケ0). ( ァ8 applefi 0208 Histology Learning System 02080ク7 Boston University/ ク7 02fi0ケ0 02fiapple080ケ0: fi fiapple0608 apple ィb ク ィB 閨0ケ (6セ404). ヲ 08 6セ ァ apple ァ ヲ ク ァ ケ ァ80ケ0 0キ30ケ0 apple03080ケ 閨 ケ0 066ァ ケ0, ケ0 00apple 鐶pple ヲ 閨03000ク ク ヲ キ ァ90キ apple08 6ァ9ィB apple キ ァ90キ ク7 apple ケ05. ヌ 020ク ァ ヌ 020ク ァ キ ッ 00apple キ307 apple ク キ307, apple 03080ク ィB ァ90ケ 閨0ケ ァ ァ8 046ヲ apple 閨0ケ0, ク708 6ァ9 apple080ク7 閨 apple 086ァ ク7 Bowman, ァ8 apple080ク7 040ク707 閨 セ4ヲフ09, apple ァ ク7 6セ404 (6ム ). ヌ ク708 6ァ9 apple ァ ケ0 020ク ァ80ケ ケ0 00apple ヲ0apple applefi 閨 ク70208apple030キ302000ク , apple086ァ ァ 閨 apple ァ ク7036ァ90キ30ケ0 applefi ク70208apple030キ302000ケ10709 apple ク7-016ァ ヌ apple ク7016ァ ァ ァ ク70208apple030キ302000ケ10709, apple080ク ク ァ ケ0 040ク707 閨 ク ク fi キ ケ0 NHERV ケ0 00ィB036ァ907090ケ0, fiapple0キ30ケ0 閨 apple ァ applefi 閨02000ケ0 02fi apple fiィB0キ ク ァ9, fi ー ケ0 ア 020ク 閨0ケ 閨0ケ apple00060キ307 apple ク キ307 (6ム ). ヲ キ3020ク ァ ケ0 apple030キ302000ケ107090ケ0 閨 apple 閨0ケ fi ケ0, apple080ク ヲ fi ァ fi0ケ0 fi ァ 閨0708. ヌ apple ク7016ァ ク 閨 ァ ケ0 020ク ァ80ケ ァ80ケ0, ケ apple fiィB0キ 閨03- MO06ヲミ ゙ メ ヌ ゙0601ヲミヲ 6ム8 メヲ O03ヲミ メ ヌ ヲ O08 09O04O メ08ヲ ヲ ゙06O ケ0 040ク 閨07090ケ0 apple030キ302000ケ107090ケ0 apple ァ apple06apple 陦0キ ク ァ9 0キ3036ァ ケ0 020キ307 apple ク キ ゙ ァ 閨02090ケ0 閨 apple 閨 ァ ケ0 apple ク7016ァ907090ケ0 0キ30ケ0 006ァ apple ク ク7-036ァ9 0ケ ッ 00apple 閨 キ30ケ0 閨 ク7 006ァ ヲ ケ ケ0 04apple ァ80ケ ケ ァ9 006ヲ ァ8 apple030キ302000ケ ケ ク708 6ァ9 0ケ0 apple ァ80ケ ケ0 020ク ァ80ケ ケ0, apple080ク7 閨 apple ァ ク ァ GLEPP1 (glomerular epithelial protein 1, apple030キ302000ケ ク7 04apple ヲ0 00apple ァ9080ク7), apple080ク7 08 6ァ apple6ァ904090ケ ク 閨 ァ ケ ァ80ケ ク7 apple ク ク fi GLEPP1 閨 apple6ァ904090ケ0 apple ァ キ30ケ0 閨 ケ0 006ァ ケ applefi ケ0 apple030キ30208apple ヲ00ケ ァ80ケ0 04apple ァ8030ク ケ apple006ァ ァ8 8. ヌ ァ8 apple ァ ケ0 020ク ァ80ケ ケ0 040ク707 閨 セ4ヲフ09 00 閨040キ3 6ヲ キ ク700apple03fi- 020キ ク70902fi ケ0, 020キ キ307 0ク ク (6ム ). O ァ907000ケ0 006ァ ァ90ケ ァ9 0ク7apple ァ90ケ0 apple080ク7 apple ク ケ0 6セ4ヲフ09 ( ァ , fi ヲ0apple080ク7 IV). ヌ ァ ァ apple 03 6ァ キ3036ァ キ307 apple ク キ307, ケ 閨 ァ ケ0 apple ァ キ307 apple apple apple fiィB0キ3, ァ キ キ キ ァ9 00apple6ァ904090ケ キ , apple0802fi apple ク キ apple030キ302000ク807080ク7036ァ9 9. O ァ ケ0 040ク707 閨080ク ク セ4ヲフ ク fi 00 閨040キ ケ apple6ァ ァ80ケ ク70ケ 閨07 046ヲ ク apple apple030キ302000ク ク キ ァ90709 (apple ァ90709, ァ90709, ク7036ァ90709, ァ ) (6ム ) ク702fi fiapple08 030ク703006ァ9- ィB080ク ク ァ ケ ァ907090ケ ク7 020ク ヲ0, ク702fi apple08apple ヲ ケ0 040ク 閨0ケ ク70ケ fi ケ ク ァ (6ム ). ヌ 6セ4ヲフ09, fi0ケ0 020キ , 040ク707 閨 apple6ァ904090ケ ク fi 020キ307 apple apple 閨040キ3 020キ ク ク , ァ90キ307 apple080ク ァ apple ァ9 0キ30ケ0 026ヲ ク ク7 040ク704026ァ ケ0 apple fi ケ ヲ0 020キ ク70ク ヌ - 0ク ク ク707 閨 ァ ァ ケ0 6セ4ヲフ09, ク ク ク707 閨 ァ ク7 020ク ヲ0, 00 閨040キ ケ0 080ク ァ907090ケ011. メ 02 ッ ァ ク70ク ヲ , 閨 ヲ apple030キ302000ク ケ0 020ク ァ80ケ ケ0, ク ケ0 (,6 5,09, ), 08 6ァ ク ク apple6ァ キ307 0ク ク ケ ァ907000ケ0 (6ム ) 4,12. 6セ

4 13 メ. メO08ヲ 06O08ヲ 6セ4O6セ4,. 03 ゙ メO09O08 O08 6ム ァ o03o096ァ9 21, 2009 E セ ァ8 apple000602fi ヲ apple apple (0909) ク ヲ00ケ ケ0 (6セ404) 00 閨040キ ク7 08apple086ァ9080ク7 040ク707 閨 ( ァ8 applefi Brenner and Rector ッs The Kidney 4 ). 026ァ8 apple ァ ケ0 020ク ァ80ケ ケ0 020キ307 apple apple 閨 ク ァ apple030キ302000ケ10709 apple08 08apple03 076ァ90709, 02090ケ0 08apple086ァ9 0ケ0 fi000キ30ケ fi03080ケ0 apple 閨 キ ケ013. O 020ク fi0ケ ァ907090ケ0 ヌ ァ ケ ク ヲ0 006ヲ003080ク70ケ0 020キ307 6セ ケ0 040ク ケ0 020キ307 apple apple セ4ヲフ09 閨 ァ ァ902000ク ク7 020ク ヲ ク7 apple ク ク7. メ ケ apple ァ8 066ァ ァ ァ9 0ク ァ ァ9, apple030800apple0803 閨 ク703 閨0ケ0 0ク ケ0 apple fi ケ0, fi ク ヲ00ケ0 07 apple ァ80ケ ケ ァ80ケ0 020キ , fi apple ケ0 0ク7 03 0ク ァ80ケ ケ0 020キ307 04apple ,15. ヌ ァ ク7 020ク ヲ0 08 6ァ ァ ケ0 04apple 閨 ケ0, ケ apple apple ヲ , applefi apple086ァ9 apple ヲ0apple02080ク apple ヲ , apple ヲ fi020ク7apple ク ク キ307, ィB ケ0 00fi0306 fiapple0キ30ケ ァ90709, apple080ク7 apple ァ9 040ク fi 020キ307 apple03086ヲ004060キ ァ ク ァ90キ ク7 020ク ヲ0 020キ307 apple apple O 020ク fi0ケ0 020キ307 apple ク キ307 apple ァ applefi 3 040ク : ク8076ァ9 06 ( ク7 7-9 nm), ァ9 06 ( 閨 ク7 10 nm) ク70ケ キ ァ ク70ケ0 ( 閨 ク7 24 nm). O キ ァ ァ9 06 apple ヲ ヲ fi fi 020キ307 apple030キ ァ804000キ ク7 020ク ヲ ケ017. O apple 閨04080ク707 閨07 apple0ク70207fi 6ァ902020ク ク ァ90キ ァ907090ケ0, apple080ク7 040ク707 閨 ク , 00 閨040キ3 020キ ァ90キ ク7 6セ404 18, セ4ヲフ09. 6セ402060ケ apple ァ 閨 apple02fi 6ァ902020ク ク ァ90キ ァ ケ0 apple , apple086ァ fi apple03080ケ ケ0 apple030キ ァ90ケ ァ ケ ァ9 000ケ ク707 閨 ク70ケ キ ァ ク70ケ0. ヌ ァ ァ キ ク ァ90キ ァ907090ケ0 006ァ G ァ90709 (globular actin, ァ ァ90709), 09 08apple086ァ9 apple08030ク ァ9ィb , ァ9ィB ケ ァ9-06 F ァ907090ケ0 (filamentous actin, ケ ァ90709). H F ァ ァ ァ9 040ク ァ8 0ク ァ apple6ァ9 apple08 fi ケ apple08030ク ヲ0, ケ ァ802080ケ ケ ケ apple0キ30ケ0 閨 ァ 閨0209 apple ク キ ァ ヲ apple6ァ9080ク7, ァ ク707 閨 apple ク708046ァ90709 II, ァ ク707 apple0208apple08 6ァ90709, 00fi apple086ァ apple ヲ ク fi ク ク ァ 閨040キ ケ ク7036ァ907090ケ ケ ァ907090ケ0 (6ム ) 20. ヌ 040ク707 apple0208apple08 6ァ ァ ァ9 apple03086ヲ apple ァ apple030キ302000ケ10709 apple080ク7 閨 ァ ィB in vitro, 02fi apple fiィB0キ3 fi キ3apple08, ク700 閨07 020ク ァ , fiapple0キ30ケ ァ902000ケ0 020キ ク キ apple ァ90ケ0 apple 閨0ケ0 020キ307 apple ク キ ク703006ァ9ィB

5 136ム ァ ァ9 21, 2009 MO06ヲミ ゙ メ ヌ ゙0601ヲミヲ 6ム8 メヲ O03ヲミ メ ヌ ヲ O08 09O04O メ08ヲ ヲ ゙06O08 E セ ァ8 apple000602fi ク7 020ク ヲ ァ907090ケ ケ apple6ァ ケ ク ァ8 apple ァ ケ0 020ク ァ80ケ ケ0. 6セ40ク ァ ケ0: S: 6セ40ク707 apple ァ90709, ゙: ァ , 6セ4ヲフ09: 6セ4apple ァ ァ : ァ90ケ0 apple 閨0ケ0 096ム8: 09030キ ァ80ケ ( ァ8 applefi Asanuma K ク ) ク703096ァ ァ9 0キ307 F ァ907090ケ0, 00 閨040キ ケ apple6ァ ァ80ケ ケ ァ ム8apple6ァ904090ケ0, ク707- 閨 ク ケ0 apple030キ302000ケ107000ケ ケ ァ80ケ0 apple ァ80ケ ケ0 020ク ァ80ケ ケ ケ0 6セ ク fi 23,24 (6ム , 6セ404. 1). 6セ400 閨 ケ0 040ク707 apple0208apple08 6ァ907090ケ0, 02 apple ヲ apple 03080ク ィB080ク fi03080キ ク7 020ク ヲ ク ァ9 020ク ァ80ケ ク704090ケ0, fi0ケ0 apple080ク ァ9ィB fi ケ0 040ク 閨07090ケ0 apple030キ302000ケ107090ケ ヲ ケ0 040ク ァ80ケ0 0ク ァ80ケ0 020キ ク ァ90キ ァ907090ケ022,25. ヌ ァ ァ ァ9 apple030キ302000ケ10709 apple080ク7 040ク fi F ァ90709, fi ケ0 apple030キ302000ケ ケ ケ0 020ク ァ80ケ ケ apple086ァ キ ァ9 0キ307 F ァ907090ケ ク7 020ク ヲ0, ク702fi ァ ケ ク703006ァ9ィB applefi ク70902 閨 キ ク ク ァ9-080ク7 Ca ケ ァ apple080ク7 020キ apple ァ ァ ヲ ァ8030キ30409 apple030キ ク807080ク7036ァ9 0ケ0, apple ク apple ケ ァ80ケ ァ80ケ0 04apple ァ8030ク ケ0, fiapple0キ30ケ0 閨 apple キ303006ァ9 applefi 閨0ケ 閨02000ケ ァ90ケ 閨0ケ キ fi 046ヲ ヲ 閨0ケ (6セ404) ヌ ァ8 apple ァ8 00apple 086ァ80ケ ヲ0 6ヲ キ307, apple ク キ ァ セ404. O06 apple ケ 閨02000ケ ァ ヲ apple6ァ9080ク7 apple 閨 セ404 0キ30ケ0 006ァ9 apple apple080ク ク ヲ ヲ ヲ apple ァ8 apple030キ302000ケ ク7 6セ404, ァ ヌ ァ

6 13 メ. メO08ヲ 06O08ヲ 6セ4O6セ4,. 03 ゙ メO09O08 O08 6ム ァ o03o096ァ9 21, 2009 ヲイ6メ2 FAT NEPH1-3 ヲュヲ ヲオヲアヲゥヲュヲァ ヲーO6メ2Oヲェヲゥヲュヲァ TCRP6 ヲェヲ ヲュヲウヲカヲ ヲアヲゥヲュヲ ヲイ ZO-1 NCK 1,2 CD2AP ヲイヲエヲュヲ ヲーヲウOヲーOヲュ6メ2ヲゥヲュヲァ Ca++ N-WASP ヲェOPTAKTINH CapZ ヲ -ヲチヲハヲモヲノヲヘ0ヒ7ヲヘヲヌ 4 ヲェヲ ヲウヲ ヲュヲゥヲュヲ ヲイ ヲェヲエヲウヲウヲ ヲアOヲーヲォヲ ヲイヲャヲ ヲェヲエヲウヲウヲ ヲアOヲイヲェヲ ヲォヲ ヲウOヲイ ヲ ヲェヲウヲゥヲュヲァヲイ 6セ セ fi ケ0 apple08036ヲ0apple ケ ァ80ケ0 046ヲ ケ ク ヲ00ケ ケ0 6セ404. ヲ 6 5 閨 ヲ0080ク ケ ヲ00ケ apple6ァ ケ ヲ0 020キ307 apple030キ302000ク ク7 6セ ク7 020ク ヲ ァ907090ケ0 006ァ ァ9 apple030キ302000ケ ヲフ 180kDa, apple080ク7 076ァ ク7036ヲ 閨 キ ケ0 08apple086ァ9 0ケ キ3020ク ァ800 apple apple ィb080ク704000ケ IgG apple 閨0ケ0 (6ム ) 30. メ0キ apple ァ applefi ァ NPHS1, ク7 08apple086ァ9080ク7 000ク7036ヲ ク 閨0ケ キ fi 046ヲ ヲ0 026ヲ0apple080ク731, apple086ァ ク7 040ク 閨07080ク ァ9080ク apple fiィB0キ ァ ィB06026ァ8 apple030キ302000ク807080ク7036ァ fi セ キ3apple08 09 apple030キ302000ク807080ク ァ ァ8 07fi04080ケ0 閨 ク ァ キ300 閨0709 閨 fi ケ ァ907090ケ0, fi ク7 mrna 02090ケ033, 34, ク 閨0709 apple030キ302000ケ10709 閨 apple ァ キ30ケ0 閨07 0ケ0 apple ケ0 006ァ ケ ケ0 020キ307 apple ク キ ァ apple セ セ fi ァ907090ケ0 apple 03080ク ィB080ク707 0ク ァ80ケ0 040ク70909 閨 ケ ヲ00ケ ァ9ィB ケ ァ8. ヲ 00010キ3020ク キ ァ90キ ァ ケ apple ク キ apple ヲ007, 040ク ケ ァ ク ァ8 046ヲ キ307 apple apple ,37, ク ク fi ケ0 040ク 閨 ァ キ307 apple03080ケ ク fi. 00apple0キ30ケ0 閨 ァ9 applefi in vitro 閨02000ケ0 ( ク 閨0ケ 閨0ケ0 apple ク キ307 apple ヲ0), ァ ァ fi apple 閨 ヲ ク7 020ク apple ケ0, ケ apple fi 0ク7apple 陦 TRPC6 (transient receptor potential ion channel 6), 08 08apple086ァ9080ケ ク ァ9 0キ30ケ ァ9 06fi07020キ307 Ca ク703006ァ9ィB ク 閨 セ キ3apple ク ァ9080ク7 apple080ク7 020キ apple ァ TRPC6 閨 ァ9 fi0206 apple ァ ァ ァ ァ8 04apple ァ8030ク ヲミ 06 6ァ ケ ァ9 0ケ0 006ァ apple6ァ ケ ァ907090ケ ク fi 02026ァ907090ケ0, 09 08apple086ァ9 00apple06-020ク 閨040キ3 006ァ9 0ケ0 apple ケ (6セ404. 1) ケ fi0ケ0 090キ3096ァ80ケ キ307 apple03080ケ ク fi 020キ , apple080ク7 閨 ァ 閨07 in vitro, キ ク7036ァ90キ ク ク ク ク ヲ ク ケ ァ907090ケ ヲ ァ ク ク ァ8 apple030キ ケ10709 Nck (non-catalytic tyrosine kinase adaptor protein). ヲ ヲ000apple apple080ク7 apple ヲ0apple ク707 閨 ク N-WASP (neuronal Wiskott-Aldrich syndrome protein), 00 閨040キ ケ0 08apple086ァ9 0ケ0 00apple ク ァ8 036ヲ キ ァ90キ ァ ケ040, , ァ apple ク fi 02026ァ907090ケ0 00 閨040キ ケ ク ケ0 046ヲ ァ80ケ ケ apple ァ CD2AP. ヌ apple ァ ァ ァ ァ8 apple030キ302000ケ ヲフ 42kDa ァ8 apple080ク7 apple ィb ク70302 閨02, ヲ ク ク ク ァ9080ク7 NPHS2, apple080ク7 006ァ ク7apple006ヲ0030ク キ apple086ァ909046ァ ケ0, apple ァ fi キ fi 046ヲ ヌ apple ァ ァ9ィB ァ ァ ァ ヲ ケ ケ0 0キ30ケ ァ

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8 13 メ. メO08ヲ 06O08ヲ 6セ4O6セ4,. 03 ゙ メO09O08 O08 6ム ァ o03o096ァ9 21, 2009 B ァ9 1. Saxen L, Lehtonen E. Embryonic kidney in organ culture. Differentation 1987; 36: Marshall B, Shankland SJ. Cell cycle regulatory proteins in podocyte health and disease. Nephron Exp Nephrol 2007; 106: Srivastava T, Garola RE, Whiting JM, Alon US. Cell cycle regulatory proteins in podocyte cell in idiopathic nephrotic syndrome of childhood. Kidney Int 2003; 63: Reiser J, Polla K. Inherited disorders of polocyte function. In: Brenner BM, ed. Brenner and Rector ッs the kidney, 8 th edition. Philadelphia, Pennsylvania: WB Saunders Company, 2007: Reiser J, Kriz W, Kreitzler M, et al. The glomerular slit diaphragm is a modified adherens junction. J Am Soc Nephrol 2000; 11: Orlando RA, Takeda T, Zak B, et al. The glomerular epithelial cell anti-adhesin podocalyxin associates with the actin cytoskeleton through interactions with ezrin. J Am Soc Nephrol 2001; 12: Achenbach J, Mengel M, Tossidou I, et al. Parietal epithelial cells in the urine as a marker of disease activity in glomerular diseases. Nephrol Dial Transplant 2008; 23: Hirakawa M, Tsuruya K, Yotsueda H, et al. Expression of synaptopodin and GLEPP1 as markers of steroid responsiveness in primary focal segmental glomerulosclerosis. Life Sci 2006; 79: Kreidberg, J.A. Functions of alpha3beta1 integrin. Curr Opin Cell Biol 2000; 12: Blattner SM, Kretzler M. Integrin-linked kinase in renal disease: connecting cell-matrix interaction to the cytoskeleton. Curr Opin Nephrol Hypertens 2005; 14: Kerjaschki D. Caught flat-footed: podocyte damage and the molecular bases of focal glomerulosclerosis. J Clin Invest 2001; 108: Yoshida T, Pan Y, Hanada H, Iwata Y, Shigekawa M. Bidirectional signaling between sarcoglycans and the integrin adhesion system in cultured L6 myocytes. J Biol Chem 1998; 273: Faul C, Asanuma K, Yanagida-Asanuma E, Kim K, Mundel P. 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Synaptopodin: an actin-associated protein in telencephalic dendrites and renal podocytes. J Cell Biol 1997; 139: 193ィC Asanuma K, Kimk K, Oh J, et al. Synaptopodin regulates the actin-bundling activity of alpha-actinin in an isoform-specific manner. J Clin Invest 2005; 115: Huber TB, Kwoh C, Wu H, et al. Bigenic mouse models of focal segmental glomerulosclerosis involving pairwise interaction of CD2AP, Fyn, and synaptopodin. J Clin Invest 2006; 116: Patrie KM, Drescher AJ, Welihinda A, Mundel P, Margolis P. Interaction of two actin-binding proteins, synaptopodin and alpha-actinin-4, with the tight junction protein MAGI-1. J Biol Chem 2002; 277: Asanuma K, Yanagida-Asanuma E, Faul C, Tomino Y, Kim K, Mundel P. Synaptopodin orchestrates actin organization and cell motility via regulation of RhoA signalling. Nat Cell Biol 2006; 8: Kaplan JM, Kim SH, North KN, et al. Mutations in ACTNA, encoding alpha-actinin-4, cause familial segmental glomerulosclerosis. Nat Genet 2000; 24: Weins A, Kenlan P, Herbert S, et al. Mutational and biological analysis of alpha-actinin-4 in focal segmental glomerulosclerosis. J Am Soc Nephrol 2005; 16: Choi HJ, Lee BH, Cho HY, et al. Familial focal segmental glomerulosclerosis associated with an ACTN4 mutation and paternal germline mosaicism. Am J Kidney Dis 2008; 51: Rodewald R, Karnovsky MJ. Porous substructure of the glomerular slit diaphragm in the rat and mouse. J Cell Biol 1974; 60: Tryggvason K. Unraveling the mechanisms of glomerular ultrafiltration: nephrin, a key component of the slit diaphragm. J Am Soc Nephrol 1999; 10: Kestila M, Lenkkeri U, Mannikko M, et al. Positionally cloned gene for a novel glomerular proteinィcnephrinィcis mutated in congenital nephrotic syndrome. Mol Cell 1998; 1: Putaala H, Soininen R, Kilpelainen P, Wartiovaara J, Tryggvason K. The murine nephrin gene is specifically expressed in kidney, brain and pancreas: Inactivation of the gene leads to massive proteinuria and neonatal death. Hum Mol Genet 2001; 10: Patrakka J, Ruotsalainen V, Ketola I, et al. Expression of nephrin in pediatric kidney diseases. J Am Soc Nephrol 2001; 12: Wang SX, Rastaldi MP, Patari A, Ahola H, Heikkila E, Holthofer H. Patterns of nephrin and a new protei- 280

9 136ム ァ ァ9 21, 2009 nuria-associated protein expression in human renal diseases. Kidney Int 2002; 61: Patari A, Forsblom C, Havana M, et al. Nephrinuria in diabetic nephropathy of type 1 diabetes. Diabetes 2003; 52: Gerke P, Huber TB, Sellin L, Benzing T, Walz G. Homodimerization and heterodimerization of the glomerular podocyte proteins nephrin and NEPH1. J Am Soc Nephrol 2003; 14: Barletta GM, Kovari IA, Verma RK, Kerjaschki D, Holzman LB. Nephrin and Neph1 co-localize at the podocyte foot process intercellular junction and form cis heterooligomers. J Biol Chem 2003; 278: Reiser J, Polu KR, M02ller CC, et al. TRPC6 is a glomerular slit diaphragm-associated channel required for normal renal function. Nat Genet 2005; 37: Winn MP, Conlon PJ, Lynn KL, et al. A mutation in the TRPC6 cation channel causes familial focal segmental glomerulosclerosis. Science 2005; 308: Jones N, Blasuting IM, Eremina V, et al. Nck adaptor proteins link nephrin to the actin cytoskeleton of kidney podocytes. Nature 2006; 440: Verma R, Kovari I, Soofi A, Nihalani D, Patrie K, Holzman LB. Nephrin ectodomain engagement results in Src kinase activation, nephrin phosphorylation, Nck recruitment, and actin polymerization. J Clin Invest 2006; 116: Boute N, Gribouval O, Roselli S, et al. NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat Genet 2000; 24: Huber TB, Kottgen M, Schilling B, Walz G, Benzing T. Interaction with podocin facilitates nephrin signaling. J Biol Chem 2001; 276: Garg P, Verma R, Nihalani D, Johnstone DB, Holzman LB. Neph1 cooperates with nephrin to transduce a signal that induces actin polymerization. Mol Cell Biol 2007: Ihalmo P, Schmid H, Rastaldi MP, et al. Expression of filtrin in human glomerular diseases. Nephrol Dial Transplant 2007; 22: Shih NY, Li J, Cotran R, Mundel P, Miner JH, Shaw AS. CD2AP localizes to the slit diaphragm and binds to nephrin via a novel C-terminal domain. Am J Pathol 2001; 159: 2303ィC Hutchings NJ, Clarkson N, Chalkley R, Barclay AN, Brown MH. Linking the T cell surface protein CD2 to MO06ヲミ ゙ メ ヌ ゙0601ヲミヲ 6ム8 メヲ O03ヲミ メ ヌ ヲ O08 09O04O メ08ヲ ヲ ゙06O08 the actin-capping protein CAPZ via CMS and CIN85. J Biol Chem 2003; 278: Lehtonen, S, Zhao F, Lehtonen E. CD2-associated protein directly interacts with the actin cytoskeleton. Am J Physiol Renal Physiol 2002; 283: Lynch DK, Zoo F, Lethonen E. A cortactin-cd2-associated protein (CD2AP) complex provides a novel link between epidermal growth factor receptor endocytosis and the actin cytoskeleton. J Biol Chem 2003; 278: Huber TB, Kwoh C, Wu H, et al. Bigenic mouse models of focal segmental glomerulosclerosis involving pairwise ineraction of CD2AP, Fyn and synaptopodin. J Clin Invest 2006; 116: Shih NY, Li J, Karpitskii V, et al. Congenital nephrotic syndrome in mice lacking CD2-associated protein. Science 1999; 286: L02wik MM, Groenen PJ, Pronk I, et al. Focal segmental glomerulosclerosis in a patient homozygous for a CD2AP mutation. Kidney Int 2007; 72: P01t01ri-Sampo A, Ihalmo P, Holth02fer H. Molecular basis of the glomerular filtration: nephrin and the emerging protein complex at the podocyte slit diaphragm. Ann Med 2006; 38: Ciani L, Patel A, Allen ND, ffrench-constant C. Mice lacking the giant protocadherin mfat1 exhibit renal slit junction abnormalities and a partially penetrant cyclopia and anophthalmia phenotype. Mol Cell Biol 2003; 23: Tanoue T, Takeichi M. New insights into Fat cadherins. J Cell Sci 2005; 118: * ァ ケ0 19/3/ apple ァ applefi apple08apple08066ァ ケ ケ0 8/7/09 A o ァ9 : メ. メ080ク ヲ ケ fi ヲ 006ァ800 縺 fi ァ908 ゙ ヲミappleapple ヲフ ァ ケ0 6セ408086ァ9 0ケ , ゙036ァ807 ヲ 0903.: Fax: k.koutroutsos@windowslive.com 281

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