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profileIhn Sik Seong, PH.D.

TitleAssistant Professor of Neurology
InstitutionMassachusetts General Hospital
DepartmentNeurology
AddressMassachusetts General Hospital
Simches Research Ctr. CPZN-5300
185 Cambridge St
Boston MA 02114
Phone617/726-5089
Fax617/643-3202
vCardDownload vCard (login for email)

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The research activities and funding listed below are automatically derived from NIH ExPORTER and other sources, which might result in incorrect or missing items. Faculty can login to make corrections and additions.
R01NS079651     (SEONG, IHN SIK)Jul 1, 2012 - Apr 30, 2016
NIH/NINDS
Effects of PolyQ Expansion on Full-length Huntingtin Protein in HD
Role: Principal Investigator

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Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Ratovitski T, O'Meally RN, Jiang M, Chaerkady R, Chighladze E, Stewart JC, Wang X, Arbez N, Roby E, Alexandris A, Duan W, Vijayvargia R, Seong IS, Lavery DJ, Cole RN, Ross CA. Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats. J Proteome Res. 2017 Aug 04; 16(8):2692-2708. PMID: 28653853.
    View in: PubMed
  2. Shin A, Shin B, Shin JW, Kim KH, Atwal RS, Hope JM, Gillis T, Leszyk JD, Shaffer SA, Lee R, Kwak S, MacDonald ME, Gusella JF, Seong IS, Lee JM. Novel allele-specific quantification methods reveal no effects of adult onset CAG repeats on HTT mRNA and protein levels. Hum Mol Genet. 2017 Apr 01; 26(7):1258-1267. PMID: 28165127.
    View in: PubMed
  3. Wehle S, Niebuhr C, Yashchenko S, Adachi I, Aihara H, Al Said S, Asner DM, Aulchenko V, Aushev T, Ayad R, Aziz T, Babu V, Bakich AM, Bansal V, Barberio E, Bartel W, Behera P, Bhuyan B, Biswal J, Bobrov A, Bondar A, Bonvicini G, Bozek A, Bracko M, Browder TE, Cervenkov D, Chang P, Chekelian V, Chen A, Cheon BG, Chilikin K, Chistov R, Cho K, Choi Y, Cinabro D, Dash N, Dingfelder J, Doležal Z, Drásal Z, Dutta D, Eidelman S, Epifanov D, Farhat H, Fast JE, Ferber T, Fulsom BG, Gaur V, Gabyshev N, Garmash A, Gillard R, Goldenzweig P, Golob B, Grzymkowska O, Guido E, Haba J, Hara T, Hayasaka K, Hayashii H, Hedges MT, Hou WS, Hsu CL, Iijima T, Inami K, Inguglia G, Ishikawa A, Itoh R, Iwasaki Y, Jacobs WW, Jaegle I, Jeon HB, Jin Y, Joffe D, Joo KK, Julius T, Kaliyar AB, Kang KH, Karyan G, Katrenko P, Kawasaki T, Kichimi H, Kiesling C, Kim DY, Kim HJ, Kim JB, Kim KT, Kim MJ, Kim SH, Kinoshita K, Koch L, Kodyš P, Korpar S, Kotchetkov D, Križan P, Krokovny P, Kuhr T, Kulasiri R, Kumita T, Kuzmin A, Kwon YJ, Lange JS, Li CH, Li L, Li Y, Li Gioi L, Libby J, Liventsev D, Lubej M, Luo T, Masuda M, Matsuda T, Miyabayashi K, Miyake H, Mizuk R, Mohanty GB, Mori T, Mussa R, Nakano E, Nakao M, Nanut T, Nath KJ, Natkaniec Z, Nayak M, Nisar NK, Nishida S, Ogawa S, Ono H, Onuki Y, Pakhlova G, Pal B, Park CS, Park CW, Park H, Paul S, Pesántez L, Piilonen LE, Pulvermacher C, Rauch J, Ritter M, Rostomyan A, Sakai Y, Sandilya S, Santelj L, Sanuki T, Sato Y, Savinov V, Schlüter T, Schneider O, Schnell G, Schwanda C, Schwartz AJ, Seino Y, Senyo K, Seon O, Seong IS, Sevior ME, Shen CP, Shibata TA, Shiu JG, Shwartz B, Simon F, Sinha R, Solovieva E, Staric M, Strube JF, Sumisawa K, Sumiyoshi T, Takizawa M, Tamponi U, Tenchini F, Trabelsi K, Tsuboyama T, Uchida M, Uglov T, Unno Y, Uno S, Urquijo P, Ushiroda Y, Usov Y, Vahsen SE, Van Hulse C, Varner G, Varvell KE, Vorobyev V, Vossen A, Waheed E, Wang CH, Wang MZ, Wang P, Watanabe M, Watanabe Y, Widmann E, Williams KM, Won E, Yamamoto H, Yamashita Y, Ye H, Yook Y, Yuan CZ, Yusa Y, Zhang ZP, Zhilich V, Zhukova V, Zhulanov V, Ziegler M, Zupanc A. Lepton-Flavor-Dependent Angular Analysis of B?K^{*}l^{+}l^{-}. Phys Rev Lett. 2017 Mar 17; 118(11):111801. PMID: 28368653.
    View in: PubMed
  4. Yang SB, Tanida K, Kim BH, Adachi I, Aihara H, Asner DM, Aulchenko V, Aushev T, Babu V, Badhrees I, Bakich AM, Barberio E, Bhardwaj V, Bhuyan B, Biswal J, Bonvicini G, Bozek A, Bracko M, Browder TE, Cervenkov D, Chekelian V, Chen A, Cheon BG, Chilikin K, Chistov R, Cho K, Chobanova V, Choi Y, Cinabro D, Dalseno J, Danilov M, Dash N, Doležal Z, Drásal Z, Dutta D, Eidelman S, Farhat H, Fast JE, Ferber T, Fulsom BG, Gabyshev N, Garmash A, Gaur V, Gillard R, Goh YM, Goldenzweig P, Greenwald D, Grygier J, Haba J, Hamer P, Hara T, Hayasaka K, Hayashii H, Hou WS, Iijima T, Inami K, Inguglia G, Ishikawa A, Itoh R, Iwasaki Y, Jacobs WW, Jaegle I, Jeon HB, Joo KK, Julius T, Kang KH, Kato E, Katrenko P, Kiesling C, Kim DY, Kim HJ, Kim JB, Kim KT, Kim MJ, Kim SH, Kim SK, Kim YJ, Kinoshita K, Kobayashi N, Kodyš P, Korpar S, Križan P, Krokovny P, Kuhr T, Kuzmin A, Kwon YJ, Lange JS, Lee IS, Li CH, Li H, Li L, Li Y, Li Gioi L, Libby J, Liventsev D, Lubej M, Masuda M, Matvienko D, Miyabayashi K, Miyata H, Mizuk R, Mohanty GB, Moll A, Moon HK, Mussa R, Nakano E, Nakao M, Nanut T, Nath KJ, Nayak M, Negishi K, Niiyama M, Nisar NK, Nishida S, Ogawa S, Okuno S, Olsen SL, Pakhlova G, Pal B, Park CW, Park H, Pedlar TK, Pestotnik R, Petric M, Piilonen LE, Pulvermacher C, Rauch J, Ritter M, Rostomyan A, Ryu S, Sahoo H, Sakai Y, Sandilya S, Santelj L, Sanuki T, Sato Y, Savinov V, Schlüter T, Schneider O, Schnell G, Schwanda C, Schwartz AJ, Seino Y, Senyo K, Seon O, Seong IS, Sevior ME, Shebalin V, Shibata TA, Shiu JG, Shwartz B, Simon F, Sohn YS, Sokolov A, Stanic S, Staric M, Stypula J, Sumihama M, Sumiyoshi T, Takizawa M, Tamponi U, Teramoto Y, Trabelsi K, Trusov V, Uchida M, Uglov T, Unno Y, Uno S, Urquijo P, Usov Y, Vanhoefer P, Varner G, Varvell KE, Vinokurova A, Vossen A, Wagner MN, Wang CH, Wang MZ, Wang P, Wang XL, Watanabe Y, Williams KM, Won E, Yamaoka J, Yashchenko S, Ye H, Yelton J, Yuan CZ, Yusa Y, Zhang ZP, Zhilich V, Zhulanov V, Zupanc A. First Observation of the Doubly Cabibbo-Suppressed Decay of a Charmed Baryon: ?_{c}^{+}?pK^{+}p^{-}. Phys Rev Lett. 2016 Jul 01; 117(1):011801. PMID: 27419562.
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  5. Garmash A, Abdesselam A, Adachi I, Aihara H, Asner DM, Aushev T, Ayad R, Aziz T, Babu V, Badhrees I, Bakich AM, Behera P, Bhardwaj V, Bhuyan B, Bobrov A, Bondar A, Bonvicini G, Bozek A, Bracko M, Browder TE, Cervenkov D, Chekelian V, Chen A, Cheon BG, Chilikin K, Cho K, Chobanova V, Choi Y, Cinabro D, Dalseno J, Danilov M, Dash N, Doležal Z, Drutskoy A, Dutta D, Eidelman S, Epifanov D, Farhat H, Fast JE, Ferber T, Fulsom BG, Gaur V, Gabyshev N, Gillard R, Goh YM, Goldenzweig P, Golob B, Hara T, Hayasaka K, Hayashii H, Iijima T, Ishikawa A, Itoh R, Iwasaki Y, Jaegle I, Joffe D, Joo KK, Julius T, Kang KH, Kato E, Kawasaki T, Kim DY, Kim JB, Kim KT, Kim MJ, Kim SH, Kim YJ, Kinoshita K, Korpar S, Križan P, Krokovny P, Kuhr T, Kuzmin A, Kwon YJ, Lange JS, Lee IS, Li C, Li H, Li L, Li Gioi L, Libby J, Liventsev D, Lukin P, Masuda M, Matvienko D, Miyabayashi K, Miyata H, Mizuk R, Mohanty GB, Moll A, Mori T, Mussa R, Nakano E, Nakao M, Nanut T, Natkaniec Z, Nishida S, Olsen SL, Pakhlov P, Pakhlova G, Pal B, Park H, Pedlar TK, Pestotnik R, Petric M, Piilonen LE, Pulvermacher C, Ribežl E, Ritter M, Rostomyan A, Sahoo H, Sakai Y, Sandilya S, Sanuki T, Savinov V, Schneider O, Schnell G, Schwanda C, Seino Y, Semmler D, Senyo K, Seong IS, Sevior ME, Shebalin V, Shen CP, Shibata TA, Shiu JG, Shwartz B, Simon F, Sohn YS, Solovieva E, Staric M, Sumiyoshi T, Tamponi U, Tanida K, Teramoto Y, Trabelsi K, Uchida M, Uehara S, Uglov T, Uno S, Van Hulse C, Vanhoefer P, Varner G, Vorobyev V, Wagner MN, Wang CH, Wang MZ, Wang P, Watanabe Y, Williams KM, Won E, Yamamoto H, Yamaoka J, Yashchenko S, Yelton J, Yook Y, Yuan CZ, Zhang ZP, Zhilich V, Zhulanov V, Zupanc A. Observation of Z_{b}(10610) and Z_{b}(10650) Decaying to B Mesons. Phys Rev Lett. 2016 May 27; 116(21):212001. PMID: 27284649.
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  6. Pal B, Schwartz AJ, Abdesselam A, Adachi I, Aihara H, Asner DM, Aushev T, Ayad R, Aziz T, Babu V, Badhrees I, Bahinipati S, Bakich AM, Barberio E, Behera P, Bhardwaj V, Bhuyan B, Biswal J, Bobrov A, Bozek A, Bracko M, Browder TE, Cervenkov D, Chekelian V, Chen A, Cheon BG, Chistov R, Cho K, Chobanova V, Choi Y, Cinabro D, Dalseno J, Dash N, Doležal Z, Drásal Z, Drutskoy A, Dutta D, Eidelman S, Farhat H, Fast JE, Fulsom BG, Gaur V, Garmash A, Gillard R, Goh YM, Goldenzweig P, Greenwald D, Grzymkowska O, Haba J, Hara T, Hayasaka K, Hayashii H, He XH, Hou WS, Inami K, Ishikawa A, Iwasaki Y, Jacobs WW, Jaegle I, Jeon HB, Joffe D, Joo KK, Julius T, Kang KH, Kato E, Kawasaki T, Kiesling C, Kim DY, Kim HJ, Kim KT, Kim MJ, Kim SH, Kinoshita K, Kodyš P, Korpar S, Križan P, Krokovny P, Kuhr T, Kumar R, Kumita T, Kuzmin A, Kwon YJ, Lee IS, Li CH, Li H, Li L, Li Gioi L, Libby J, Liventsev D, Lukin P, Luo T, Masuda M, Matvienko D, Miyabayashi K, Miyata H, Mizuk R, Mohanty GB, Mohanty S, Moll A, Moon HK, Mori T, Mussa R, Nakano E, Nakao M, Nanut T, Natkaniec Z, Nayak M, Nisar NK, Nishida S, Ogawa S, Okuno S, Pakhlov P, Pakhlova G, Park CW, Park H, Paul S, Pedlar TK, Pesántez L, Pestotnik R, Petric M, Piilonen LE, Pulvermacher C, Rauch J, Ribežl E, Ritter M, Rostomyan A, Ryu S, Sahoo H, Sakai Y, Sandilya S, Sanuki T, Sato Y, Savinov V, Schlüter T, Schneider O, Schnell G, Schwanda C, Seino Y, Senyo K, Seon O, Seong IS, Shebalin V, Shibata TA, Shiu JG, Shwartz B, Simon F, Sohn YS, Sokolov A, Solovieva E, Stanic S, Staric M, Stypula J, Sumihama M, Sumiyoshi T, Tamponi U, Teramoto Y, Trabelsi K, Uchida M, Uehara S, Uglov T, Uno S, Urquijo P, Usov Y, Van Hulse C, Vanhoefer P, Varner G, Vinokurova A, Vossen A, Wagner MN, Wang CH, Wang MZ, Wang XL, Watanabe M, Watanabe Y, Williams KM, Won E, Yamaoka J, Yelton J, Yuan CZ, Yusa Y, Zhang ZP, Zhilich V, Zhulanov V, Zupanc A. Observation of the Decay B_{s}^{0}?K^{0}K[over ¯]^{0}. Phys Rev Lett. 2016 Apr 22; 116(16):161801. PMID: 27152790.
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  7. Vijayvargia R, Epand R, Leitner A, Jung TY, Shin B, Jung R, Lloret A, Singh Atwal R, Lee H, Lee JM, Aebersold R, Hebert H, Song JJ, Seong IS. Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function. Elife. 2016 Mar 22; 5:e11184. PMID: 27003594; PMCID: PMC4846397.
  8. Jaegle I, Adachi I, Aihara H, Al Said S, Asner DM, Aushev T, Ayad R, Bakich AM, Bansal V, Barrett M, Bhuyan B, Bozek A, Bracko M, Browder TE, Cervenkov D, Chang MC, Cheon BG, Chilikin K, Cho K, Chobanova V, Choi SK, Choi Y, Cinabro D, Dalseno J, Doležal Z, Drásal Z, Drutskoy A, Dutta D, Eidelman S, Epifanov D, Farhat H, Fast JE, Ferber T, Frost O, Gaur V, Gabyshev N, Ganguly S, Garmash A, Getzkow D, Gillard R, Goh YM, Golob B, Grzymkowska O, Hayasaka K, Hayashii H, He XH, Hedges M, Hou WS, Iijima T, Inami K, Ishikawa A, Iwasaki Y, Julius T, Kang KH, Kato E, Kawasaki T, Kim DY, Kim JB, Kim JH, Kim SH, Kinoshita K, Ko BR, Kodyš P, Korpar S, Križan P, Krokovny P, Kuzmin A, Kwon YJ, Lange JS, Lee IS, Lewis P, Li Gioi L, Libby J, Liventsev D, Matvienko D, Miyata H, Mizuk R, Mohanty GB, Moll A, Mussa R, Nakano E, Nakao M, Nisar NK, Nishida S, Ogawa S, Pakhlov P, Pakhlova G, Park H, Pedlar TK, Pesántez L, Petric M, Piilonen LE, Ritter M, Rostomyan A, Sakai Y, Sandilya S, Santelj L, Sanuki T, Sato Y, Savinov V, Schneider O, Schnell G, Schwanda C, Semmler D, Senyo K, Seon O, Seong I, Sevior ME, Shebalin V, Shibata TA, Shiu JG, Shwartz B, Simon F, Sinha R, Sohn YS, Staric M, Sumihama M, Sumisawa K, Tamponi U, Tatishvili G, Teramoto Y, Thorne F, Uchida M, Uehara S, Unno Y, Uno S, Vahsen SE, Van Hulse C, Vanhoefer P, Varner G, Vinokurova A, Wagner MN, Wang CH, Wang MZ, Wang P, Wang XL, Watanabe M, Watanabe Y, Williams KM, Won E, Yamaoka J, Yashchenko S, Yook Y, Yusa Y, Zhilich V, Zhulanov V, Zupanc A. Search for the dark photon and the dark Higgs boson at belle. Phys Rev Lett. 2015 May 29; 114(21):211801. PMID: 26066427.
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  9. Biagioli M, Ferrari F, Mendenhall EM, Zhang Y, Erdin S, Vijayvargia R, Vallabh SM, Solomos N, Manavalan P, Ragavendran A, Ozsolak F, Lee JM, Talkowski ME, Gusella JF, Macdonald ME, Park PJ, Seong IS. Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation. Hum Mol Genet. 2015 May 01; 24(9):2442-57. PMID: 25574027; PMCID: PMC4383859.
  10. Quintero-Rivera F, Xi QJ, Keppler-Noreuil KM, Lee JH, Higgins AW, Anchan RM, Roberts AE, Seong IS, Fan X, Lage K, Lu LY, Tao J, Hu X, Berezney R, Gelb BD, Kamp A, Moskowitz IP, Lacro RV, Lu W, Morton CC, Gusella JF, Maas RL. MATR3 disruption in human and mouse associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus. Hum Mol Genet. 2015 Apr 15; 24(8):2375-89. PMID: 25574029; PMCID: PMC4380077.
  11. Galkina EI, Shin A, Coser KR, Shioda T, Kohane IS, Seong IS, Wheeler VC, Gusella JF, Macdonald ME, Lee JM. HD CAGnome: a search tool for huntingtin CAG repeat length-correlated genes. PLoS One. 2014; 9(4):e95556. PMID: 24751919; PMCID: PMC3994101.
  12. Lee JM, Galkina EI, Levantovsky RM, Fossale E, Anne Anderson M, Gillis T, Srinidhi Mysore J, Coser KR, Shioda T, Zhang B, Furia MD, Derry J, Kohane IS, Seong IS, Wheeler VC, Gusella JF, MacDonald ME. Dominant effects of the Huntington's disease HTT CAG repeat length are captured in gene-expression data sets by a continuous analysis mathematical modeling strategy. Hum Mol Genet. 2013 Aug 15; 22(16):3227-38. PMID: 23595883; PMCID: PMC3723309.
  13. Kim HG, Kim HT, Leach NT, Lan F, Ullmann R, Silahtaroglu A, Kurth I, Nowka A, Seong IS, Shen Y, Talkowski ME, Ruderfer D, Lee JH, Glotzbach C, Ha K, Kjaergaard S, Levin AV, Romeike BF, Kleefstra T, Bartsch O, Elsea SH, Jabs EW, MacDonald ME, Harris DJ, Quade BJ, Ropers HH, Shaffer LG, Kutsche K, Layman LC, Tommerup N, Kalscheuer VM, Shi Y, Morton CC, Kim CH, Gusella JF. Translocations disrupting PHF21A in the Potocki-Shaffer-syndrome region are associated with intellectual disability and craniofacial anomalies. Am J Hum Genet. 2012 Jul 13; 91(1):56-72. PMID: 22770980; PMCID: PMC3397276.
  14. Fossale E, Seong IS, Coser KR, Shioda T, Kohane IS, Wheeler VC, Gusella JF, MacDonald ME, Lee JM. Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative. Hum Mol Genet. 2011 Nov 01; 20(21):4258-67. PMID: 21840924; PMCID: PMC3188996.
  15. Jacobsen JC, Gregory GC, Woda JM, Thompson MN, Coser KR, Murthy V, Kohane IS, Gusella JF, Seong IS, MacDonald ME, Shioda T, Lee JM. HD CAG-correlated gene expression changes support a simple dominant gain of function. Hum Mol Genet. 2011 Jul 15; 20(14):2846-60. PMID: 21536587; PMCID: PMC3118763.
  16. Reis SA, Thompson MN, Lee JM, Fossale E, Kim HH, Liao JK, Moskowitz MA, Shaw SY, Dong L, Haggarty SJ, MacDonald ME, Seong IS. Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis. Hum Mol Genet. 2011 Jun 15; 20(12):2344-55. PMID: 21447599; PMCID: PMC3098733.
  17. Seong IS, Woda JM, Song JJ, Lloret A, Abeyrathne PD, Woo CJ, Gregory G, Lee JM, Wheeler VC, Walz T, Kingston RE, Gusella JF, Conlon RA, MacDonald ME. Huntingtin facilitates polycomb repressive complex 2. Hum Mol Genet. 2010 Feb 15; 19(4):573-83. PMID: 19933700; PMCID: PMC2807366.
  18. Rho SH, Park HH, Kang GB, Im YJ, Kang MS, Lim BK, Seong IS, Seol J, Chung CH, Wang J, Eom SH. Crystal structure of Bacillus subtilis CodW, a noncanonical HslV-like peptidase with an impaired catalytic apparatus. Proteins. 2008 May 01; 71(2):1020-6. PMID: 17979190.
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  19. Kim HG, Kishikawa S, Higgins AW, Seong IS, Donovan DJ, Shen Y, Lally E, Weiss LA, Najm J, Kutsche K, Descartes M, Holt L, Braddock S, Troxell R, Kaplan L, Volkmar F, Klin A, Tsatsanis K, Harris DJ, Noens I, Pauls DL, Daly MJ, MacDonald ME, Morton CC, Quade BJ, Gusella JF. Disruption of neurexin 1 associated with autism spectrum disorder. Am J Hum Genet. 2008 Jan; 82(1):199-207. PMID: 18179900; PMCID: PMC2253961.
  20. Lee JM, Ivanova EV, Seong IS, Cashorali T, Kohane I, Gusella JF, MacDonald ME. Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism. PLoS Genet. 2007 Aug; 3(8):e135. PMID: 17708681; PMCID: PMC1950164.
  21. Seong IS, Ivanova E, Lee JM, Choo YS, Fossale E, Anderson M, Gusella JF, Laramie JM, Myers RH, Lesort M, MacDonald ME. HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum Mol Genet. 2005 Oct 01; 14(19):2871-80. PMID: 16115812.
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  22. Park E, Rho YM, Koh OJ, Ahn SW, Seong IS, Song JJ, Bang O, Seol JH, Wang J, Eom SH, Chung CH. Role of the GYVG pore motif of HslU ATPase in protein unfolding and translocation for degradation by HslV peptidase. J Biol Chem. 2005 Jun 17; 280(24):22892-8. PMID: 15849200.
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  23. Gines S, Ivanova E, Seong IS, Saura CA, MacDonald ME. Enhanced Akt signaling is an early pro-survival response that reflects N-methyl-D-aspartate receptor activation in Huntington's disease knock-in striatal cells. J Biol Chem. 2003 Dec 12; 278(50):50514-22. PMID: 14522959.
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  24. Kang MS, Kim SR, Kwack P, Lim BK, Ahn SW, Rho YM, Seong IS, Park SC, Eom SH, Cheong GW, Chung CH. Molecular architecture of the ATP-dependent CodWX protease having an N-terminal serine active site. EMBO J. 2003 Jun 16; 22(12):2893-902. PMID: 12805205; PMCID: PMC162141.
  25. Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, Wheeler VC, Persichetti F, MacDonald ME. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet. 2003 Mar 01; 12(5):497-508. PMID: 12588797.
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  26. Seong IS, Kang MS, Choi MK, Lee JW, Koh OJ, Wang J, Eom SH, Chung CH. The C-terminal tails of HslU ATPase act as a molecular switch for activation of HslV peptidase. J Biol Chem. 2002 Jul 19; 277(29):25976-82. PMID: 12011053.
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  27. Wang J, Song JJ, Seong IS, Franklin MC, Kamtekar S, Eom SH, Chung CH. Nucleotide-dependent conformational changes in a protease-associated ATPase HsIU. Structure. 2001 Nov; 9(11):1107-16. PMID: 11709174.
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  28. Cho EG, Kim MG, Kim C, Kim SR, Seong IS, Chung C, Schwartz RH, Park D. N-terminal processing is essential for release of epithin, a mouse type II membrane serine protease. J Biol Chem. 2001 Nov 30; 276(48):44581-9. PMID: 11567025.
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  29. Kang MS, Lim BK, Seong IS, Seol JH, Tanahashi N, Tanaka K, Chung CH. The ATP-dependent CodWX (HslVU) protease in Bacillus subtilis is an N-terminal serine protease. EMBO J. 2001 Feb 15; 20(4):734-42. PMID: 11179218; PMCID: PMC145431.
  30. Wang J, Song JJ, Franklin MC, Kamtekar S, Im YJ, Rho SH, Seong IS, Lee CS, Chung CH, Eom SH. Crystal structures of the HslVU peptidase-ATPase complex reveal an ATP-dependent proteolysis mechanism. Structure. 2001 Feb 07; 9(2):177-84. PMID: 11250202.
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  31. Seong IS, Oh JY, Lee JW, Tanaka K, Chung CH. The HslU ATPase acts as a molecular chaperone in prevention of aggregation of SulA, an inhibitor of cell division in Escherichia coli. FEBS Lett. 2000 Jul 21; 477(3):224-9. PMID: 10908725.
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  32. Seong IS, Oh JY, Yoo SJ, Seol JH, Chung CH. ATP-dependent degradation of SulA, a cell division inhibitor, by the HslVU protease in Escherichia coli. FEBS Lett. 1999 Jul 30; 456(1):211-4. PMID: 10452560.
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  33. Lee CS, Seong IS, Song HK, Chung CH, Suh SW. Crystallization and preliminary X-ray crystallographic analysis of the protease inhibitor ecotin in complex with chymotrypsin. Acta Crystallogr D Biol Crystallogr. 1999 May; 55(Pt 5):1091-2. PMID: 10216315.
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  34. Han SJ, Jung PM, Kim H, Kim JE, Hong J, Hwang EH, Seong I. Multiple intestinal ulcerations and perforations secondary to methicillin-resistant Staphylococcus aureus enteritis in infants. J Pediatr Surg. 1999 Mar; 34(3):381-6. PMID: 10211636.
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  35. Oh JY, Eun YM, Yoo SJ, Seol JH, Seong IS, Lee CS, Chung CH. LonR9 carrying a single Glu614 to Lys mutation inhibits the ATP-dependent protease La (Lon) by forming mixed oligomeric complexes. Biochem Biophys Res Commun. 1998 Sep 08; 250(1):32-5. PMID: 9735326.
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  36. Yoo SJ, Kim HH, Shin DH, Lee CS, Seong IS, Seol JH, Shimbara N, Tanaka K, Chung CH. Effects of the cys mutations on structure and function of the ATP-dependent HslVU protease in Escherichia coli. The Cys287 to Val mutation in HslU uncouples the ATP-dependent proteolysis by HslvU from ATP hydrolysis. J Biol Chem. 1998 Sep 04; 273(36):22929-35. PMID: 9722513.
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  37. Kim KI, Woo KM, Seong IS, Lee ZW, Baek SH, Chung CH. Mutational analysis of the two ATP-binding sites in ClpB, a heat shock protein with protein-activated ATPase activity in Escherichia coli. Biochem J. 1998 Aug 01; 333 ( Pt 3):671-6. PMID: 9677327; PMCID: PMC1219631.
  38. Yoo SJ, Seol JH, Seong IS, Kang MS, Chung CH. ATP binding, but not its hydrolysis, is required for assembly and proteolytic activity of the HslVU protease in Escherichia coli. Biochem Biophys Res Commun. 1997 Sep 18; 238(2):581-5. PMID: 9299555.
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  39. Yoo SJ, Shim YK, Seong IS, Seol JH, Kang MS, Chung CH. Mutagenesis of two N-terminal Thr and five Ser residues in HslV, the proteolytic component of the ATP-dependent HslVU protease. FEBS Lett. 1997 Jul 21; 412(1):57-60. PMID: 9257689.
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  40. Seong IS, Lee CS, Park HK, Suh SW, Kang MS, Chung CH. Mutational analysis for the role of C-terminal region of ecotin, a dimeric inhibitor of pancreatic serine proteases. Biochem Mol Biol Int. 1997 Jul; 42(4):799-807. PMID: 19856297.
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  41. Shin DH, Song HK, Seong IS, Lee CS, Chung CH, Suh SW. Crystal structure analyses of uncomplexed ecotin in two crystal forms: implications for its function and stability. Protein Sci. 1996 Nov; 5(11):2236-47. PMID: 8931142; PMCID: PMC2143284.
  42. Seong IS, Lee HR, Seol JH, Park SK, Lee CS, Suh SW, Hong YM, Kang MS, Ha DB, Chung CH. The P1 reactive site methionine residue of ecotin is not crucial for its specificity on target proteases. A potent inhibitor of pancreatic serine proteases from Escherichia coli. J Biol Chem. 1994 Aug 26; 269(34):21915-8. PMID: 8063835.
    View in: PubMed
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