浠婂ぉ鏄細

瀹為獙瀹よ祫璁綉

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

瀹為獙瀹よ祫璁綉鏃堕棿:2018-08-12 鐐瑰嚮: 娆?/span> 鐧惧害鎼滅储

銆愬璇汇€?/strong>鏂戒竴鍏粍锛氫汉婧怭KD1鍜孭KD2澶嶅悎鐗╃殑缁撴瀯 鍦⊿tructure of the human PKD1/PKD2 complex鏂囩珷涓紝鏂戒竴鍏粍涓撴敞鐨勬槸涓€绉嶅父瑙侀仐浼犵梾甯告煋鑹蹭綋鏄炬€ч仐浼犲鍥婅偩鐥咃紙ADPKD锛夈€傝繖涓€鐤剧梾涓昏鍏宠仈涓や釜鍩哄洜绐佸彉锛屽嵆pkd1鍜宲kd2銆傜爺绌跺洟闃熼娆℃姤閬撲簡澶氬泭鑲剧梾鐩稿叧铔嬬櫧PKD1鍜孭KD2澶嶅悎鐗╂暣浣撹繎鍘熷瓙鍒嗚鲸鐜囩殑鍐峰喕鐢甸暅缁撴瀯銆?甯告煋鑹蹭綋鏄炬€ч仐......
TAG鏍囩锛?棰滃畞 Science 鏂戒竴鍏?/a> 缁撴瀯鐢熺墿瀛?/a>
鏂戒竴鍏粍锛氫汉婧怭KD1鍜孭KD2澶嶅悎鐗╃殑缁撴瀯

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

銆€銆€鍦?ldquo;Structure of the human PKD1/PKD2 complex”鏂囩珷涓紝鏂戒竴鍏粍涓撴敞鐨勬槸涓€绉嶅父瑙侀仐浼犵梾——甯告煋鑹蹭綋鏄炬€ч仐浼犲鍥婅偩鐥咃紙ADPKD锛夈€傝繖涓€鐤剧梾涓昏鍏宠仈涓や釜鍩哄洜绐佸彉锛屽嵆pkd1鍜宲kd2銆傜爺绌跺洟闃熼娆℃姤閬撲簡澶氬泭鑲剧梾鐩稿叧铔嬬櫧PKD1鍜孭KD2澶嶅悎鐗╂暣浣撹繎鍘熷瓙鍒嗚鲸鐜囩殑鍐峰喕鐢甸暅缁撴瀯銆?/p>

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

銆€銆€甯告煋鑹蹭綋鏄炬€ч仐浼犲鍥婅偩鐥呮槸鎱㈡€ц偩鑴忕梾鐨勯噸瑕佽鍥犱箣涓€锛屽彂鐥呯巼涓?/400-1/1000锛屽叏鐞冪害1200涓囨偅鑰呮繁鍙楄繖涓€鐤剧梾鐨勫奖鍝嶃€傜害50%鎮h€呬細鍙戝睍鍒扮粓鏈湡鑲惧姛鑳借“绔紝闇€瑕佽繘琛屽紓婧愯偩鑴忕Щ妞嶆垨鑰呯粓韬娑查€忔瀽娌荤枟銆傛垜鍥界害鏈?50涓囨鐤剧梾鎮h€咃紝姣忓勾閮芥湁鏁颁互涓囪鐨勬偅鑰呰嫤鑻︾瓑寰呮棤鍋挎崘鐚殑鑲炬簮鎴栬€呴€氳繃鏃犱紤姝㈢殑閫忔瀽缁存寔鐢熷懡銆侫DPKD涓嶄粎缁欐偅鑰呴€犳垚涓ラ噸鐨勮韩浣撳拰绮剧涓婄殑鎶樼(锛屽悓鏃剁粰鎮h€呭搴甫鏉ユ矇閲嶇殑缁忔祹璐熸媴銆?/p>

銆€銆€浜烘簮pkd1鍩哄洜瀹氫綅浜?6鍙锋煋鑹蹭綋锛岀紪鐮佷簡闀垮害涓?302涓皑鍩洪吀鍖呭惈11娆¤法鑶滆灪鏃嬬殑铔嬬櫧PKD1銆傝€岀敱pkd2缂栫爜鐨凱KD2铔嬬櫧鏄疨KD1鐨勪即渚e垎瀛愶紝瀵筆KD1鐨勬姌鍙犮€佸湪缁嗚優鍣ㄩ棿鐨勮浆杩愩€佸拰铔嬬櫧鎴愮啛鍏锋湁鏋佸叾閲嶈鐨勪綔鐢ㄣ€?/p>

銆€銆€鏂戒竴鍏粍鍒╃敤鍐峰喕鐢甸暅鎶€鏈紝鑾峰緱鎴煭鐨勪汉绫籔KD1/PKD2澶嶅悎浣撶粨鏋勶紝鍒嗚鲸鐜囪揪鍒?.6 Å銆備粬浠彂鐜帮紝PKD1鍜孭KD2铔嬬櫧浼氬舰鎴愪竴涓嫭鐗圭殑涓€姣斾笁澶嶅悎鐗?( 1 PKD1: 3 PKD2 )銆傚叿浣撹€岃█锛孭KD1鍖呭惈涓€涓數鍘嬮棬鎺ф€х瀛愰€氶亾锛圴GIC锛夛紝閫氳繃涓嶱KD2鐩镐簰浣滅敤褰㈡垚涓€涓潪瑙勮寖鐨凾RP閫氶亾浣撶郴缁撴瀯銆傝繖涓€杩囩▼涓紝PKD1涓殑S6铻烘棆浼氬湪涓棿鏂锛屽舰鎴怱6a鍜孲6b涓ゆ銆傞櫎浜哣GIC涔嬪锛孭KD1杩樻湁涓€涓?-TM缁撴瀯鍩熷拰涓€涓優璐≒LAT缁撴瀯鍩熴€?/p>

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

鍥?锛欰.浜烘簮PKD1鍜孭KD2铔嬬櫧鐨勬嫇鎵戠粨鏋勭ず鎰忓浘銆侭.浜烘簮PKD1鍜孭KD2铔嬬櫧澶嶅悎鐗╃粨鏋勶紱C.浜烘簮PKD1鐙壒鐨勯€氶亾缁撴瀯鍩熴€傦紙鍥剧墖鏉ユ簮锛氭竻鍗庡ぇ瀛︾敓鍛界瀛﹀闄級

銆€銆€杩欎簺缁撴瀯鐨勮В鏋愪负PKD铔嬬櫧鍔熻兘鐨勭爺绌跺缓绔嬩簡涓€涓鏋讹紝骞惰繘涓€姝ヤ负甯告煋鑹蹭綋鏄炬€у鍥婅偩鐥呯殑鐥呭洜浠ュ強娌荤枟瀵圭瓥鎻愪緵鏂扮殑绾跨储銆?/p>

銆€銆€棰滃畞缁勶細浜烘簮Ptch1铔嬬櫧鐨勫喎鍐荤數闀滅粨鏋?/strong>

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

銆€銆€Hedgehog ( Hh )淇″彿閫氳矾鍦ㄨ儦鑳庡彂鑲蹭腑寰堥噸瑕侊紝瀹冪殑杩囧害娲昏穬涓庣檶鐥囨湁鍏炽€傝閫氳矾鐨勬牳蹇冩槸涓€绉嶈绉颁负Patched 1 (Ptch1) 鐨勮啘鍙椾綋銆傚畠闂存帴鎶戝埗涓€绉嶇О涓篠moothened鐨凣铔嬬櫧鍋惰仈鍙椾綋銆傚綋Ptch1缁撳悎鍒嗘硨鐨勮泲鐧紿h鏃讹紝杩欑鎶戝埗寰楀埌缂撹В锛岄€氳矾鎵撳紑銆?/p>

銆€銆€棰滃畞璇鹃缁勬姤閬撲簡浜虹被Ptch1鐨勫喎鍐荤數闀滅粨鏋勶紝Ptch1鍒嗗埆浠?.9 Å鍜?.6 Å鐨勫垎杈ㄧ巼涓庝汉绫籗onic hedgehog ( ShhN )鐨凬鏈缁撴瀯鍩熷鍚堬紱鍒╃敤杩欎簺鍐峰喕鐢甸暅缁撴瀯锛岃璇鹃缁勮瀵熷埌浜哖tch1鐨?2涓法鑶滅粨鏋勫煙浠ュ強涓や釜缁嗚優澶栫粨鏋勫煙ECD1涓嶦CD2銆?/p>

涓ょ瘒Science锛佹柦涓€鍏€侀瀹佽棰樼粍鍙戣〃鈥滅粨鏋勭敓鐗╁鈥濇柊鎴愭灉

銆€銆€姝ゅ锛屼粬浠繕鍙戠幇涓ょ缁撴瀯涓兘瀛樺湪涓ょ绫诲浐閱囩姸鐨勫瘑搴︼紝閫氳繃杩涗竴姝ョ粨鏋勫鍚戠殑绐佸彉鍒嗘瀽琛ㄦ槑锛孲hhN鍜孭tch1涔嬮棿鐨勭浉浜掍綔鐢ㄤ緷璧栦簬绫诲浐閱囥€傜被鍥洪唶缁撳悎缂洪櫡Ptch1绐佸彉浣撶殑缁撴瀯鏄剧ず鏄庢樉鐨勬瀯璞¢噸鎺掋€?/p>

銆€銆€浜虹被Ptch1鍙婂叾涓嶴hhN鐨勫鍚堢墿鐨勭粨鏋勬彮绀轰簡Ptch1鍜孲hhN涔嬮棿璇嗗埆鐨勫垎瀛愬熀纭€锛涘悓鏃禤tch1涓袱涓被鍥洪唶缁撳悎浣嶇偣鐨勯壌瀹氫篃涓烘湭鏉ョ爺绌禜h閫氳矾寤虹珛浜嗛噸瑕佺殑妗嗘灦銆?/p> 锛堟湰鏂囨潵婧愶細鐢熺墿鎺㈢储 锛?

(璐d换缂栬緫锛氬ぇ鏋?

寮曠敤鍦板潃锛?a href="#" target=_blank>

TAG鏍囩锛?棰滃畞 Science 鏂戒竴鍏?/a> 缁撴瀯鐢熺墿瀛?/a>
鍙戣〃璇勮
璇疯嚜瑙夐伒瀹堜簰鑱旂綉鐩稿叧鐨勬斂绛栨硶瑙勶紝涓ョ鍙戝竷鑹叉儏銆佹毚鍔涖€佸弽鍔ㄧ殑瑷€璁恒€?/small>
璇勪环:
琛ㄦ儏:
鏈€鏂拌瘎璁?/strong> 杩涘叆璇︾粏璇勮椤?gt;>
鎺ㄨ崘鍐呭