Slowed photoresponse recovery and age-related degeneration in cones lacking G protein-coupled receptor kinase 1.

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L, Tappaz ML, Fety R, Vincent M, Renaud B, Sassard J, Kobayashi H, Magnoni MS, Govoni S, Izumi F, Wada A, Trabucchi M, Vaccari A, Sinzinger H, Firbas W, De Marte ML, Enesco HE, Haibach H, Hosler MW, Sartin JL, Lamperti AA, Corder CN, Bixenstine KJ, Shapiro AP, Riehl W, Medvedev ZA, Medvedeva MN, Crowne HM, Kendall MD, Luthy IA, Calandra RS, Simpson BJ, Bedi KS, Sanyal S, De Ruiter A, Dees C, Seidel CL, Allen JC, Mukhopadhyay A, Mato M, Ookawara S, Sugamata M, Aikawa E, Harasawa R, Mitsuoka T, Cano J, Machado A, Brown SJ, Ramachandran M, Iyer GY, Sharma R, Patnaik SK, Syrovy I, Mato M, Mato TK, Smit JD, Guggenheim R, Bauer PG, Barbanel G, Assenmacher I, Planz G, Bieber J, Mencke HJ, von Bernuth G, Oroszlan G, Lakatos L, Szabo L, Matkovics B, Karmazsin L, Schlager G, Freeman R, Heim ME, Taylor KB, Nickla H, Anderson J, Palzkill T, Blois R, Feinberg I, Gaillard JM, Kupfer DJ, Webb WB, Matsuzawa T, Hatsugai M, Allen SR, Stahelin HB, Seiler WO, Spiegel R, Alba P, Ferres MD, Xamena N, Creus A, Marcos R, Parker VD, Soliman KF, Seshadri R, Banerji AP, Shah PN, Mezzetti G, Moruzzi MS, Barbiroli B, Lescoat G, Schroder D, Hehmke B, Zuhlke H, Kaur H, Shrivastava PK, Kobayashi Y, Yoshimitsu Y, Okahata S, Usui T, von Hahn HP, Lisy V, Stastny F, Murphy S, Hajkova B, Mato M, Ookawara S, Sano M, Fukuda S, Singh SM, Wang CH, Phillips A, Bartosz G, Matsuzawa T, Cinader B, Scanes CG, Camaratto A, Ventura MA, Schapiro H, Hotta SS, Outten WE, Klein AW, Matsuzawa T, Cinader B, Bartosz G, Michalak W, Gondko R, Singh SM, Phillips A, Wang CH, Solomon HF, Hopper AF, Salminen A, Kainulainen H, Vihko V, Pieri C, Giuli C, Bertoni-Freddari C, Piantanelli L, Nagy IZ, Blumenthal SJ, McConnaughey MM, Iams SG, Wapnir RA, Mancusi VJ, Goldstein LA, Senault C, Solier M, Beauvallet M, Portet R, Crispens CG Jr, Nagai K, Murano S, Minokoshi Y, Okuda H, Kinutani M, Bartosz G, Grzelinska E, Wagner J, Scaro JL, Miranda C, Martin BM, Carrera M, Misra CH, Chase D, Ullrick WC, Dasse KA, Burke DJ, Goldberg AH, von Hahn HP, Robert L, Meier-Ruge W, Macieira-Coelho A, Lints FA, Frolkis VV, Fabris N, Schlettwein-Gsell D, Rokyta R, Mares P, Sobotka P, Jakab L, Penzes L, Bartosz G, Swierczynski B, Gondko R, Bartosz G, Bartkowiak A, Kiselev GV, Minenko AN, Moritz V, Oehme P, Nehlig A, Lehr PR, Shoemaker DD, Dietrick DD, Cysyk RL, Vento R, Taibi G, Tesoriere L, Tesoriere G, Costa FF, Zago MA, Bottura C, Altura BM, Lum G, Turlapaty PD, Altura BT, Matkovics B, Lakatos L, Szabo L, Karmazsin L, Jamieson DD, Taylor KM, Frithz G, Ronquist G, Andersson B, Moskalewski S, Golaszewska A, Ksiazek T, Chanda S, Patra PB, Chanda R, Deb C, Kashiwamata S, Aono S, Semba RK, Smokovitis A, Binder BR, Yong LC, Prema AK, Mohamed UV, Bounias M, Chen PS, Shah PG, Sheth AR, Frowein A, Lapin V, Yaeger Y, Nathan I, Dvilansky A, Meyerstein N, Friend JP, Meserve LA, Belledonne M, Preuss JM, Preuss HG, David JC, Math F, Davrainville JL, Kato T, Hama T, Nagatsu T, Kuzuya H, Sakakibara S, Tsui E, Yeung D, Toh YC, Kundu SN, De Adhikari H, Bhattacharyya BK, Bhattacharyya SP, Maeda N, Kon K, Sekiya M, Shiga T, Gilmore SA, Waddington JL, MacCulloch MJ, Sambrooks JE, Lapointe G, Nosal G, Mohsin A, Baker GT, Zschunke RE, Podgorski EM Jr, Ramakrishnan CV, Bhandari SD, Ioppolo C, Currell DL, Civalleri L, Antonini E, Shafa F, Sato H, Yamamoto K, Kamei C, Shimizu M, Mohsin A, Richer C, Venturini-Souto N, Giudicelli JF, Nelson K, Daniel WL, Jones E, Davies JE, Hughes RE, Seshadri RS, Morley AA, Trainor KJ, Sorrell J, Yates MS, Hiley CR, Gosden RG, Fowler RE, Stopp M, Hartwich R, Braunlich H, Bartosz G, Tannert C, Fried R, Leyko W, Tajima T, Iijima K, Watanabe T, Giri KV, Singh SN, Rusic M, Levental M, Rakic L, Karen P, Pohlova I, Jelinek J, Sugimoto K, Sato T, Hazama H, Kunimoto N, Maes J, Gerber GB, Weiler J, Sachsse W, Weinshilboum RM, Dunnette J, Raymond F, Kleinberg F, Nagatsu T, Kato T, Hashimoto Y, Numata Y, Yamori Y, Okamoto K, Clausell DE, Soliman KF, Ostadalova I, Babicky A, Obenberger J, Kelly SS, Petrelli F, Coderoni S, Moretti P, Marsili G, Paparelli M, Arfellini G, Prodi G, Grilli S, Asotra K, Malhotra RK, Matsuno T, Shirasawa N, Umino Y, Katow S, Shishido A, Miller SC, Kaiserman M, Osmond DG, Danieli Betto D, Midrio M, Pierpaoli W, Nukari-Siltovuori A, Utili R, Abernathy CO, Aron SA, Zimmerman HJ, Zolla L, Ioppolo C, Amiconi G, Benaglia A, Antonini E, Chen CS, Gates GR, Martinovitch PN, Zivkovic N, Pavic D, Cimmino CC, Djaczenko W, Zanussi C, Rugarli C, Perussia B, Smeraldi RS, Foppen FH, Liuzzi A, Kopin IJ, Everett GM, Blumenthal SG, Ruebner BH, Ikeda RM, Hanson FW, Bergstrom DE, Siebers JW, Schmidtke J, Engel W, Nagatsu T, Kato T, Numata Y, Ikuta K, Sano M, Nagatsu I, Takeuchi T, Matsuzaki M, Umezawa H, Hoff HG, Ghraf R, Lax ER, Schriefers H, Goswami MN, Dainat J, Rebiere, Hosey MM, Green RD, Baker GT, Schmidt T, Meffert H, Diezel W, Sonnichsen N, Kouvelas ED, Savakis CE, Tzebelikos ET, Bonatsos G, Mitrossilis S, Loehr KD, Sayyadi P, Lamprecht I, Cohet YA, David JR, Csaba G, Nagy SU, Yew DT, Nigg HN, Svoboda JA, Thompson MJ, Dutky SR, Kaplanis JN, Robbins WE, Bocci V, Lipetz J, Boswell RE, Kuzuya H, Ikeno T, Ikeno K, Kato T, Nagatsu T, Qureshi F, Allegretti N, Marusic M, Benedetti A, Baker RS, Kato T, Ikuta K, Takahashi K, Nagatsu T, Catalan RE, Vila T, Castillon MP, Erlinger M, Schlatterer B, Mohan S, Radha E, Verzar F, Strittmatter-Ackerschott E, Harley PR, Muller D, Kersten L, Ankermann H, Maura G, Vaccari A, Kriesten K, Zimmermann E, Sommer H, Egge H, Hensel W, Hauschteck-Jungen E, Cohet Y, David J, Gianfranceschi GL, Amici D, Guglielmi L, Amici D, Gianfranceschi GL, Marsili G, Michetti L, Story JA, Kritchevsky D, Aschheim P, Gordon HA, Doberauer W, Gianfranceschi GL, Amici D, Guglielmi L, Hofecker G, Kment A, Niedermuruller H, Robert L, Moczar M, Robert M, Piantamelli L, Ermini M, Ricciotti R, Amici D, Gianfranceschi GL, Marsili G, Michetti L, Martinovitch PN, Ivanisevic-Milovanovic, Milicic AF, Martinovic JV, Manfredi M, Ermini M, Honorati MC, Ermini M, Edington DW, McCafferty WB, Chronister RB, Bernstein JJ, Zornetzer SF, White LE Jr, Singh SN, Ganzoni AM, Spati B, Buhler H, Buhlmann H, Tonkin SY, Firkin BG, Pierpaoli W, Sorkin E, Viidik A, Ermini M, Szelenyi I, Cornelius E, Vyskocil F, Gutmann E, Chatterji S, Wall JC, Jeffrey JW, Sobel H, Bowman R, Comolli R, Hajdu A, Rona G, Dalderup LM, Visser W, Silberberg R, Lesker P, Siegenthaler PA, Benz G, Richard A, Pilet PE, Tuchweber B, Bozzini CE, Barrio Rendo ME, Kofoed JA, Meier C, Singh SN, Kanungo MS, Seigenthaler PA, Mohanty MK, Patnaik BK, Del Cerro MP, Snider RS, Oster M, Jelinek J, Krecek J, Ermini M, Verzar F, Krishnan RS, Spadoni MA, Ticca M, Bocci V, Klinge O, Stocker E, Morin RJ, Teller W, Kruger C, Rockstein M, Srivastava PN, van Oortmerssen GA, Beardmore JA, Bernardis LL, Weihe WH, Bernardis LL, Bahorsky M, Bohacek L, Skelton FR, Bernardis LL, Fox MW, Mende TJ, Fischer R, Deyl Z, Ehrlichova E, Jasmin G, GOULLET P, KAUFMANN K, Hahn P, Drahota Z, Koldovsky O, De Martino C, Tonietti G, Accinni L, Fachini G, Gianfranceschi G, van den Berg CJ, van Kempen GM, Sorokin C, Heikkinen E, Kulonen E, ALTSCHULER H, LIEBERSON M, SPITZER JJ, MANOCHA SL, SHARMA SP, KOBRLE V, CHVAPIL M, KATO R, CHIESARA E, FRONTINO G, FARNER D, VERZAR F, BROCAS J, VERZAR F, RABADJIJA L, VRANIC M, ALLEGRETTI N, POKRAJAC N, BRUCKNER R, GSELL O, HUGIN F, BATSCHELET E, VERZAR F, SCHNEIDER JA, DI MACCO G, OLBRICH O, WOODFORD-WILLIAMS E, OLBRICH O, WOODFORD-WILLIAMS E, FISHER RB, RECHENBERGER J, SCHULZE W, THUNG PJ, NOCKER J, SCHULZ FH, ANTONINI FM, GREPPI E, SCARDIGLI G, SALVINI L, GUIDI G, SCHETTLER G, BAUR H, JESSOP WJ, FLEISCHER K, DIVRY P, BURGER M, HUGIN F, VERZAR F, FREYDBERG-LUCAS V, VERZAR F, BRULL L, KEIL C, MUHLBOCK O, THUNG PJ, BOURNE GH, DANIELLI JF, JOSEPH NR, BOURLIERE F, VERZAR F, BANGA I, BALO J, SZABO D, KUHN W, MIESCHER K, VERZAR F, VERZAR F, Milgram NW, Araujo JA, Hagen TM, Treadwell BV, Ames BN, Glenn JV, Beattie JR, Barrett L, Frizzell N, Thorpe SR, Boulton ME, McGarvey JJ, Stitt AW, Bai XY, Chen X, Sun AQ, Feng Z, Hou K, Fu B, Vernace VA, Arnaud L, Schmidt-Glenewinkel T, Figueiredo-Pereira ME, Robinet A, Millart H, Oszust F, Hornebeck W, Bellon G, Costin GE, Hearing VJ, Zarse K, Schulz TJ, Birringer M, Ristow M, Llorens JV, Navarro JA, Martinez-Sebastian MJ, Baylies MK, Schneuwly S, Botella JA, Molto MD, Zangarelli A, Chanseaume E, Morio B, Brugere C, Mosoni L, Rousset P, Giraudet C, Patrac V, Gachon P, Boirie Y, Walrand S, Tahara EB, Barros MH, Oliveira GA, Netto LE, Kowaltowski AJ, Cyr M, Sotnikova TD, Gainetdinov RR, Caron MG, Bjork K, Saarikoski ST, Arlinde C, Kovanen L, Osei-Hyiaman D, Ubaldi M, Reimers M, Hyytia P, Heilig M, Sommer WH, Mathews CK, Lesnefsky EJ, He D, Moghaddas S, Hoppel CL, Aquilano K, Vigilanza P, Rotilio G, Ciriolo MR, Vigetti D, Moretto P, Viola M, Genasetti A, Rizzi M, Karousou E, Pallotti F, De Luca G, Passi A, Sanz A, Caro P, Ayala V, Portero-Otin M, Pamplona R, Barja G, Ding Q, Dimayuga E, Markesbery WR, Keller JN, Maurelli R, Zambruno G, Guerra L, Abbruzzese C, Dimri G, Gellini M, Bondanza S, Dellambra E, Arck PC, Overall R, Spatz K, Liezman C, Handjiski B, Klapp BF, Birch-Machin MA, Peters EM, Broome CS, Kayani AC, Palomero J, Dillmann WH, Mestril R, Jackson MJ, McArdle A, Wang X, Hockerman GH, Green HW 3rd, Babbs CF, Mohammad SI, Gerrard D, Latour MA, London B, Hannon KM, Pond AL, Ventura N, Rea SL, Handerson ST, Condo I, Testi R, Johnson TE, Fasanaro P, Magenta A, Zaccagnini G, Cicchillitti L, Fucile S, Eusebi F, Biglioli P, Capogrossi MC, Martelli F, Yang X, Doser TA, Fang CX, Nunn JM, Janardhanan R, Zhu M, Sreejayan N, Quinn MT, Ren J, Bilsland LG, Dick JR, Pryce G, Petrosino S, Di Marzo V, Baker D, Greensmith L, Bolin CM, Basha R, Cox D, Zawia NH, Maloney B, Lahiri DK, Cardozo-Pelaez F, Rasmussen BB, Fujita S, Wolfe RR, Mittendorfer B, Roy M, Rowe VL, Volpi E, Ballard VL, Sharma A, Duignan I, Holm JM, Chin A, Choi R, Hajjar KA, Wong SC, Edelberg JM, Kwak HB, Song W, Lawler JM, Hsieh CC, Papaconstantinou J, Razzaque MS, Sitara D, Taguchi T, St-Arnaud R, Lanske B, Yu J, Lei L, Liang Y, Hinh L, Hickey RP, Huang Y, Liu D, Yeh JL, Rebar E, Case C, Spratt K, Sessa WC, Giordano FJ, Jin L, Liu T, Lagoda GA, Champion HC, Bivalacqua TJ, Burnett AL, Ritz BW, Lelkes PI, Gardner EM, Noyan-Ashraf MH, Wu L, Wang R, Juurlink BH, Vazquez-Manrique RP, Gonzalez-Cabo P, Ros S, Aziz H, Baylis HA, Palau F, Oka S, Liu W, Masutani H, Hirata H, Shinkai Y, Yamada S, Yoshida T, Nakamura H, Yodoi J, Basha MR, Murali M, Siddiqi HK, Ghosal K, Siddiqi OK, Lashuel HA, Ge YW, Lahiri DK, Zawia NH, Castello L, Froio T, Cavallini G, Biasi F, Sapino A, Leonarduzzi G, Bergamini E, Poli G, Chiarpotto E, Dudek EJ, Shang F, Valverde P, Liu Q, Hobbs M, Taylor A, McColl G, Vantipalli MC, Lithgow GJ, Umeda S, Suzuki MT, Okamoto H, Ono F, Mizota A, Terao K, Yoshikawa Y, Tanaka Y, Iwata T, von Bohlen und Halbach O, Minichiello L, Unsicker K, Hepple RT, Baker DJ, Kaczor JJ, Krause DJ, Godbout JP, Chen J, Abraham J, Richwine AF, Berg BM, Kelley KW, Johnson RW, Marneros AG, Olsen BR, Dip R, Naegeli H, Piec I, Listrat A, Alliot J, Chambon C, Taylor RG, Bechet D, Zhao L, Tong T, Zhang Z, Perez GI, Jurisicova A, Matikainen T, Moriyama T, Kim MR, Takai Y, Pru JK, Kolesnick RN, Tilly JL, Ferrington DA, Husom AD, Thompson LV, Phillips T, Leeuwenburgh C, Judge S, Jang YM, Smith A, Hagen T, Leeuwenburgh C, Agrawal AK, Shapiro BH, Descamps FJ, Martens E, Proost P, Starckx S, Van den Steen PE, Van Damme J, Opdenakker G, Cuthbertson D, Smith K, Babraj J, Leese G, Waddell T, Atherton P, Wackerhage H, Taylor PM, Rennie MJ, Hamada K, Vannier E, Sacheck JM, Witsell AL, Roubenoff R, Von Kobbe C, May A, Grandori C, Bohr VA, Zhang HJ, Doctrow SR, Xu L, Oberley LW, Beecher B, Morrison J, Oberley TD, Kregel KC, Casanova ML, Bravo A, Martinez-Palacio J, Fernandez-Acenero MJ, Villanueva C, Larcher F, Conti CJ, Jorcano JL, Cooper TM, Mockett RJ, Sohal BH, Sohal RS, Orr WC, Guillet C, Prod'homme M, Balage M, Gachon P, Giraudet C, Morin L, Grizard J, Boirie Y, Johnson GB, Riggs BL, Platt JL, Tuo J, Smith BC, Bojanowski CM, Meleth AD, Gery I, Csaky KG, Chew EY, Chan CC, Louzada PR, Lima AC, Mendonca-Silva DL, Noel F, De Mello FG, Ferreira ST, Rimbert V, Boirie Y, Bedu M, Hocquette JF, Ritz P, Morio B, Jin K, Mao XO, Cottrell B, Schilling B, Xie L, Row RH, Sun Y, Peel A, Childs J, Gendeh G, Gibson BW, Greenberg DA, Radak Z, Chung HY, Naito H, Takahashi R, Jung KJ, Kim HJ, Goto S, McKiernan SH, Bua E, McGorray J, Aiken J, Bua E, McKiernan SH, Aiken JM, Morrow G, Samson M, Michaud S, Tanguay RM, Stuart JA, Karahalil B, Hogue BA, Souza-Pinto NC, Bohr VA, McArdle A, Dillmann WH, Mestril R, Faulkner JA, Jackson MJ, Higami Y, Pugh TD, Page GP, Allison DB, Prolla TA, Weindruch R, Poch E, Carbonell P, Franco S, Diez-Juan A, Blasco MA, Andres V, Zou Y, Jung KJ, Kim JW, Yu BP, Chung HY, Sung S, Yao Y, Uryu K, Yang H, Lee VM, Trojanowski JQ, Pratico D, Alzghoul MB, Gerrard D, Watkins BA, Hannon K, Zhang HJ, Xu L, Drake VJ, Xie L, Oberley LW, Kregel KC, Greco M, Villani G, Mazzucchelli F, Bresolin N, Papa S, Attardi G, Merker K, Ullrich O, Schmidt H, Sitte N, Grune T, Walker GA, Thompson FJ, Brawley A, Scanlon T, Devaney E, Damy T, Ratajczak P, Robidel E, Bendall JK, Oliviero P, Boczkowski J, Ebrahimian T, Marotte F, Samuel JL, Heymes C, Murakami S, Salmon A, Miller RA, Nahoum V, Gangloff A, Shi R, Lin SX, de Cavanagh EM, Piotrkowski B, Basso N, Stella I, Inserra F, Ferder L, Fraga CG, Bussolati B, Deambrosis I, Russo S, Deregibus MC, Camussi G, Asencio C, Rodriguez-Aguilera JC, Ruiz-Ferrer M, Vela J, Navas P, Csiszar A, Ungvari Z, Koller A, Edwards JG, Kaley G, Shimokawa I, Higami Y, Tsuchiya T, Otani H, Komatsu T, Chiba T, Yamaza H, Escames G, Leon J, Macias M, Khaldy H, Acuna-Castroviejo D, Tuo J, Jaruga P, Rodriguez H, Bohr VA, Dizdaroglu M, Plackett TP, Schilling EM, Faunce DE, Choudhry MA, Witte PL, Kovacs EJ, Forster MJ, Morris P, Sohal RS, Veitonmaki N, Fuxe J, Hultdin M, Roos G, 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Adv Exp Med Biol. 2006;572:133-9.

A growing number of human neurodegenerative diseases are associated with the expression of misfolded proteins that oligomerize and form aggregate structures. Over time, accumulation of misfolded proteins leads to the disruption of cellular protein folding homeostasis and eventually to cellular dysfunction and death. To investigate the relationship between misfolded proteins, neuropathology and aging, we have developed models utilizing the nematode C. elegans. In addition to being genetically tractable, C. elegans have rapid growth rates and short life-cycles, providing unique advantages for modeling neurodegenerative diseases of aging caused by the stress of misfolded proteins. The C. elegans models described here express polyglutamine expansion-containing proteins, as occur in Huntington's disease. Through the use of tissue-specific expression of different lengths of fluorescently tagged polyglutamine repeats, we have examined the dynamics of aggregate formation both within individual cells and over time throughout the lifetime of individual animals, identifying aging and other genetic modifiers as an important physiologic determinant of aggregation and toxicity.

This abstract at PubMed.