| Makoto Urushitani, Samer Abou Ezzi, Akinori Matsuo, Ikuo Tooyama, Jean-Pierre Julien |
| The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS. |
| FASEB J 2008 July, 22: 2476-2487. PMID: 18337461 |
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| 彫朎懱-僑儖僕暘斿宯傊偺曄堎SOD1偺桝憲偲傾僌儕僎乕僩宍惉偼壠懓惈嬝堔弅惈懁嶕峝壔徢偺昦懺偵娭梌偡傞
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亂梫巪亃
攚宨乯丂壠懓惈嬝堔弅惈懁嶕峝壔徢乮ALS乯偺20%偵僗乕僷乕僆僉僔僪僕僗儉僞乕僛1 (superoxide dismutase 1; SOD1)偺撍慠曄堎偑懚嵼偟傑偡偑丄曄堎SOD1偵傛傞慖戰揑塣摦僯儏乕儘儞巰偺儊僇僯僘儉偼埶慠晄柧偱偡丅変乆偼埲慜丄嵶朎幙僞儞僷僋偱偁傞曄堎SOD1偑恄宱暘斿僞儞僷僋偱偁傞僋儘儌僌儔僯儞偲寢崌偟偰嵶朎奜暘斿偝傟傞偙偲丄嵶朎奜偺曄堎SOD1偑儈僋儘僌儕傾偺妶惈壔傗塣摦僯儏乕儘儞巰傪婲偙偡偙偲傪敪尒偟(Urushtiani et al, Nat Neurosci 2006)丄偝傜偵嵶朎奜偺曄堎SOD1傪昗揑偲偟偨柶塽椕朄偵惉岟偟傑偟偨(Urushitani et al, PNAS 2007)丅偟偐偟丄杮棃嵶朎幙僞儞僷僋偱偁傞SOD1偺嵶朎撪嬊嵼偵偮偄偰偼堦掕偺尒夝偑摼傜傟偰偍傜偢丄暘斿宯偵擖傞婡彉傕晄柧偱偟偨丅
栚揑偲曽朄乯丂崱夞丄嵶朎幙僞儞僷僋偱偁傞SOD1偑彫朎懱乮ER乯-僑儖僕暘斿宯偐傜暘斿偝傟傞暘巕儊僇僯僘儉傪夝柧偡傞偨傔丄攟梴嵶朎傪巊偭偨live cell imaging, 曄堎SOD1僩儔儞僗僕僃僯僢僋儅僂僗偺慻怐傪巊偭偨僞儞僷僋壔妛揑幚尡丄偝傜偵儔僢僩娞憻儈僋儘僜乕儉暘夋傪梡偄偨in vitro translocation幚尡傪峴偄傑偟偨丅
寢壥乯丂EGFP昗幆傪偟偨SOD1乮栰惗宆丄曄堎宆乯傪恄宱宯偺晄巰壔嵶朎乮neuro2a嵶朎乯偵堚揱巕摫擖偟丄24帪娫屻ATP偺懚嵼壓偵僕僊僩僯儞張棟傪偟偰嵶朎幙僞儞僷僋傪wash-out偟偨屻偵嫟徟揰儗乕僓乕尠旝嬀偱娤嶡偡傞偲丄栰惗宆丒曄堎宆偲傕偵SOD1偑彫朎條峔憿撪偵巆懚偟偰偍傝丄僑儖僕儅乕僇乕偲偺嫟嬊嵼傪帵偟傑偟偨丅偝傜偵曄堎SOD1僩儔儞僗僕僃僯僢僋儅僂僗偺愐悜儔僀僙乕僩傪丄僗僋儘乕僗枾搙岡攝墦怱暘棧朄偲丄iodoxanol傪梡偄偨Floating挻墦怱暘棧朄偵傛偭偰SOD1偑ER-Golgi暘夋偵偁傞偙偲傪徹柧偟傑偟偨丅傑偨丄嵶朎惉暘暘夋偟偨愐悜儔僀僙乕僩傪chemical cross-linking偟偨屻SOD1峈懱傪梡偄偰Western blotting偟偨偲偙傠丄曄堎SOD1傪娷傓傾僌儕僎乕僩偑儈僋儘僜乕儉暘夋偱嵟傕挊柧偱偁傞偙偲傪敪尒偟傑偟偨丅偝傜偵慻傒姺偊SOD1僞儞僷僋偑摵/垷墧偑攝埵偟側偄傾億忬懺偱丄ATP懚嵼壓偱儔僢僩娞憻儈僋儘僜乕儉偵庢傝崬傑傟傞偙偲傪帵偟傑偟偨丅
寢榑乯丂埲忋偺寢壥偼儈僗僼僅乕儖僪偟偨曄堎SOD1偑ER-Golgi暘斿宯偵擖傞偙偲偲ALS偺昦懺偵娭梌偡傞偙偲傪惗壔妛偵徹柧偟偨傕偺偱丄ALS偺昦懺偺棟夝丄帯椕朄奐敪偵婑梌偡傞傕偺偲峫偊傜傟傑偡丅
暥愑丂暘巕恄宱壢妛尋媶僙儞僞乕丂幗扟丂恀
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