Joan S Steffan

Title(s)Recall Faculty, Psychiatry
SchoolSchool of Medicine
Address1001 Health Sciences Road
Irvine CA 92697-3950
Phone(949) 824-6365
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    Pathogenic impact of Huntingtin phosphorylation in Huntington's Disease
    NIH/NINDS R56NS072453Apr 1, 2012 - Jun 30, 2018
    Role: Principal Investigator
    Pathogenic impact of Huntington phosphorylation in Huntington's Disease
    NIH/NINDS R01NS072453Apr 1, 2012 - Mar 31, 2016
    Role: Principal Investigator

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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Huntingtin contains an ubiquitin-binding domain and regulates lysosomal targeting of mitochondrial and RNA-binding proteins. Proc Natl Acad Sci U S A. 2024 Aug 06; 121(32):e2319091121. Fote GM, Eapen VV, Lim RG, Yu C, Salazar L, McClure NR, McKnight J, Nguyen TB, Heath MC, Lau AL, Villamil MA, Miramontes R, Kratter IH, Finkbeiner S, Reidling JC, Paulo JA, Kaiser P, Huang L, Housman DE, Thompson LM, Steffan JS. PMID: 39074279.
      View in: PubMed   Mentions: 1     Fields:    Translation:HumansAnimalsCells
    2. Aberrant splicing in Huntington's disease via disrupted TDP-43 activity accompanied by altered m6A RNA modification. bioRxiv. 2023 Nov 02. Nguyen TB, Miramontes R, Chillon-Marinas C, Maimon R, Vazquez-Sanchez S, Lau AL, McClure NR, England WE, Singha M, Stocksdale JT, Jang KH, Jung S, McKnight JI, Ho LN, Faull RLM, Steffan JS, Reidling JC, Jang C, Lee G, Cleveland DW, Lagier-Tourenne C, Spitale RC, Thompson LM. PMID: 37961595; PMCID: PMC10635028.
      View in: PubMed   Mentions:
    3. Author Correction: Gene expression and functional deficits underlie TREM2-knockout microglia responses in human models of Alzheimer's disease. Nat Commun. 2023 Mar 02; 14(1):1194. McQuade A, Kang YJ, Hasselmann J, Jairaman A, Sotelo A, Coburn M, Shabestari SK, Chadarevian JP, Fote G, Tu CH, Danhash E, Silva J, Martinez E, Cotman C, Prieto GA, Thompson LM, Steffan JS, Smith I, Davtyan H, Cahalan M, Cho H, Blurton-Jones M. PMID: 36864071; PMCID: PMC9981556.
      View in: PubMed   Mentions:    Fields:    
    4. APOE4 dysregulates autophagy in cultured cells. Autophagy Rep. 2022; 1(1):29-33. Fote GM, Steffan JS. PMID: 38912292; PMCID: PMC11192451.
      View in: PubMed   Mentions:
    5. Isoform-dependent lysosomal degradation and internalization of apolipoprotein E requires autophagy proteins. J Cell Sci. 2022 01 15; 135(2). Fote GM, Geller NR, Efstathiou NE, Hendricks N, Vavvas DG, Reidling JC, Thompson LM, Steffan JS. PMID: 34982109; PMCID: PMC8917355.
      View in: PubMed   Mentions: 9     Fields:    Translation:AnimalsCells
    6. Diminished LC3-Associated Phagocytosis by Huntington's Disease Striatal Astrocytes. J Huntingtons Dis. 2022; 11(1):25-33. Wakida NM, Lau AL, Nguyen J, Cruz GMS, Fote GM, Steffan JS, Thompson LM, Berns MW. PMID: 35253772; PMCID: PMC9028675.
      View in: PubMed   Mentions: 5     Fields:    Translation:AnimalsCells
    7. Cooperation of cell adhesion and autophagy in the brain: Functional roles in development and neurodegenerative disease. Matrix Biol Plus. 2021 Dec; 12:100089. Hernandez SJ, Fote G, Reyes-Ortiz AM, Steffan JS, Thompson LM. PMID: 34786551; PMCID: PMC8579148.
      View in: PubMed   Mentions: 7  
    8. Transglutaminase 6 Is Colocalized and Interacts with Mutant Huntingtin in Huntington Disease Rodent Animal Models. Int J Mol Sci. 2021 Aug 18; 22(16). Schulze-Krebs A, Canneva F, Stemick J, Plank AC, Harrer J, Bates GP, Aeschlimann D, Steffan JS, von Hörsten S. PMID: 34445621; PMCID: PMC8396294.
      View in: PubMed   Mentions: 1     Fields:    Translation:AnimalsCells
    9. Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1. Autophagy. 2021 Jan; 17(1):1-382. Klionsky DJ, Abdel-Aziz AK, Abdelfatah S, Abdellatif M, Abdoli A, Abel S, Abeliovich H, Abildgaard MH, Abudu YP, Acevedo-Arozena A, Adamopoulos IE, Adeli K, Adolph TE, Adornetto A, Aflaki E, Agam G, Agarwal A, Aggarwal BB, Agnello M, Agostinis P, Agrewala JN, Agrotis A, Aguilar PV, Ahmad ST, Ahmed ZM, Ahumada-Castro U, Aits S, Aizawa S, Akkoc Y, Akoumianaki T, Akpinar HA, Al-Abd AM, Al-Akra L, Al-Gharaibeh A, Alaoui-Jamali MA, Alberti S, Alcocer-Gómez E, Alessandri C, Ali M, Alim Al-Bari MA, Aliwaini S, Alizadeh J, Almacellas E, Almasan A, Alonso A, Alonso GD, Altan-Bonnet N, Altieri DC, Álvarez ÉMC, Alves S, Alves da Costa C, Alzaharna MM, Amadio M, Amantini C, Amaral C, Ambrosio S, Amer AO, Ammanathan V, An Z, Andersen SU, Andrabi SA, Andrade-Silva M, Andres AM, Angelini S, Ann D, Anozie UC, Ansari MY, Antas P, Antebi A, Antón Z, Anwar T, Apetoh L, Apostolova N, Araki T, Araki Y, Arasaki K, Araújo WL, Araya J, Arden C, Arévalo MA, Arguelles S, Arias E, Arikkath J, Arimoto H, Ariosa AR, Armstrong-James D, Arnauné-Pelloquin L, Aroca A, Arroyo DS, Arsov I, Artero R, Asaro DML, Aschner M, Ashrafizadeh M, Ashur-Fabian O, Atanasov AG, Au AK, Auberger P, Auner HW, Aurelian L, Autelli R, Avagliano L, Ávalos Y, Aveic S, Aveleira CA, Avin-Wittenberg T, Aydin Y, Ayton S, Ayyadevara S, Azzopardi M, Baba M, Backer JM, Backues SK, Bae DH, Bae ON, Bae SH, Baehrecke EH, Baek A, Baek SH, Baek SH, Bagetta G, Bagniewska-Zadworna A, Bai H, Bai J, Bai X, Bai Y, Bairagi N, Baksi S, Balbi T, Baldari CT, Balduini W, Ballabio A, Ballester M, Balazadeh S, Balzan R, Bandopadhyay R, Banerjee S, Banerjee S, Bánréti Á, Bao Y, Baptista MS, Baracca A, Barbati C, Bargiela A, Barilà D, Barlow PG, Barmada SJ, Barreiro E, Barreto GE, Bartek J, Bartel B, Bartolome A, Barve GR, Basagoudanavar SH, Bassham DC, Bast RC, Basu A, Batoko H, Batten I, Baulieu EE, Baumgarner BL, Bayry J, Beale R, Beau I, Beaumatin F, Bechara LRG, Beck GR, Beers MF, Begun J, Behrends C, Behrens GMN, Bei R, Bejarano E, Bel S, Behl C, Belaid A, Belgareh-Touzé N, Bellarosa C, Belleudi F, Belló Pérez M, Bello-Morales R, Beltran JSO, Beltran S, Benbrook DM, Bendorius M, Benitez BA, Benito-Cuesta I, Bensalem J, Berchtold MW, Berezowska S, Bergamaschi D, Bergami M, Bergmann A, Berliocchi L, Berlioz-Torrent C, Bernard A, Berthoux L, Besirli CG, Besteiro S, Betin VM, Beyaert R, Bezbradica JS, Bhaskar K, Bhatia-Kissova I, Bhattacharya R, Bhattacharya S, Bhattacharyya S, Bhuiyan MS, Bhutia SK, Bi L, Bi X, Biden TJ, Bijian K, Billes VA, Binart N, Bincoletto C, Birgisdottir AB, Bjorkoy G, Blanco G, Blas-Garcia A, Blasiak J, Blomgran R, Blomgren K, Blum JS, Boada-Romero E, Boban M, Boesze-Battaglia K, Boeuf P, Boland B, Bomont P, Bonaldo P, Bonam SR, Bonfili L, Bonifacino JS, Boone BA, Bootman MD, Bordi M, Borner C, Bornhauser BC, Borthakur G, Bosch J, Bose S, Botana LM, Botas J, Boulanger CM, Boulton ME, Bourdenx M, Bourgeois B, Bourke NM, Bousquet G, Boya P, Bozhkov PV, Bozi LHM, Bozkurt TO, Brackney DE, Brandts CH, Braun RJ, Braus GH, Bravo-Sagua R, Bravo-San Pedro JM, Brest P, Bringer MA, Briones-Herrera A, Broaddus VC, Brodersen P, Brodsky JL, Brody SL, Bronson PG, Bronstein JM, Brown CN, Brown RE, Brum PC, Brumell JH, Brunetti-Pierri N, Bruno D, Bryson-Richardson RJ, Bucci C, Buchrieser C, Bueno M, Buitrago-Molina LE, Buraschi S, Buch S, Buchan JR, Buckingham EM, Budak H, Budini M, Bultynck G, Burada F, Burgoyne JR, Burón MI, Bustos V, Büttner S, Butturini E, Byrd A, Cabas I, Cabrera-Benitez S, Cadwell K, Cai J, Cai L, Cai Q, Cairó M, Calbet JA, Caldwell GA, Caldwell KA, Call JA, Calvani R, Calvo AC, Calvo-Rubio Barrera M, Camara NO, Camonis JH, Camougrand N, Campanella M, Campbell EM, Campbell-Valois FX, Campello S, Campesi I, Campos JC, et al. PMID: 33634751; PMCID: PMC7996087.
      View in: PubMed   Mentions: 986     Fields:    Translation:HumansAnimalsCells
    10. PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington's disease. Proc Natl Acad Sci U S A. 2021 01 26; 118(4). Morozko EL, Smith-Geater C, Monteys AM, Pradhan S, Lim RG, Langfelder P, Kachemov M, Kulkarni JA, Zaifman J, Hill A, Stocksdale JT, Cullis PR, Wu J, Ochaba J, Miramontes R, Chakraborty A, Hazra TK, Lau A, St-Cyr S, Orellana I, Kopan L, Wang KQ, Yeung S, Leavitt BR, Reidling JC, Yang XW, Steffan JS, Davidson BL, Sarkar PS, Thompson LM. PMID: 33468657; PMCID: PMC7848703.
      View in: PubMed   Mentions: 17     Fields:    Translation:HumansAnimalsCells
    11. Gene expression and functional deficits underlie TREM2-knockout microglia responses in human models of Alzheimer's disease. Nat Commun. 2020 10 23; 11(1):5370. McQuade A, Kang YJ, Hasselmann J, Jairaman A, Sotelo A, Coburn M, Shabestari SK, Chadarevian JP, Fote G, Tu CH, Danhash E, Silva J, Martinez E, Cotman C, Prieto GA, Thompson LM, Steffan JS, Smith I, Davtyan H, Cahalan M, Cho H, Blurton-Jones M. PMID: 33097708; PMCID: PMC7584603.
      View in: PubMed   Mentions: 100     Fields:    Translation:AnimalsCells
    12. Serine residues 13 and 16 are key modulators of mutant huntingtin induced toxicity in Drosophila. Exp Neurol. 2021 04; 338:113463. Chatterjee M, Steffan JS, Lukacsovich T, Marsh JL, Agrawal N. PMID: 32941796.
      View in: PubMed   Mentions: 3     Fields:    Translation:HumansAnimalsCells
    13. IKKβ slows Huntington's disease progression in R6/1 mice. Proc Natl Acad Sci U S A. 2019 05 28; 116(22):10952-10961. Ochaba J, Fote G, Kachemov M, Thein S, Yeung SY, Lau AL, Hernandez S, Lim RG, Casale M, Neel MJ, Monuki ES, Reidling J, Housman DE, Thompson LM, Steffan JS. PMID: 31088970; PMCID: PMC6561205.
      View in: PubMed   Mentions: 16     Fields:    Translation:AnimalsCells
    14. Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models. J Huntingtons Dis. 2018; 7(2):137-150. Franich NR, Basso M, André EA, Ochaba J, Kumar A, Thein S, Fote G, Kachemov M, Lau AL, Yeung SY, Osmand A, Zeitlin SO, Ratan RR, Thompson LM, Steffan JS. PMID: 29843246; PMCID: PMC6002862.
      View in: PubMed   Mentions: 10     Fields:    Translation:Animals
    15. Longitudinal Biochemical Assay Analysis of Mutant Huntingtin Exon 1 Protein in R6/2 Mice. J Huntingtons Dis. 2018; 7(4):321-335. Morozko EL, Ochaba J, Hernandez SJ, Lau A, Sanchez I, Orellana I, Kopan L, Crapser J, Duong JH, Overman J, Yeung S, Steffan JS, Reidling J, Thompson LM. PMID: 30452420; PMCID: PMC6294605.
      View in: PubMed   Mentions: 3     Fields:    Translation:HumansAnimals
    16. Human Neural Stem Cell Transplantation Rescues Functional Deficits in R6/2 and Q140 Huntington's Disease Mice. Stem Cell Reports. 2018 01 09; 10(1):58-72. Reidling JC, Relaño-Ginés A, Holley SM, Ochaba J, Moore C, Fury B, Lau A, Tran AH, Yeung S, Salamati D, Zhu C, Hatami A, Cepeda C, Barry JA, Kamdjou T, King A, Coleal-Bergum D, Franich NR, LaFerla FM, Steffan JS, Blurton-Jones M, Meshul CK, Bauer G, Levine MS, Chesselet MF, Thompson LM. PMID: 29233555; PMCID: PMC5768890.
      View in: PubMed   Mentions: 42     Fields:    Translation:HumansAnimalsCells
    17. Human Neural Progenitor Transplantation Rescues Behavior and Reduces α-Synuclein in a Transgenic Model of Dementia with Lewy Bodies. Stem Cells Transl Med. 2017 06; 6(6):1477-1490. Goldberg NRS, Marsh SE, Ochaba J, Shelley BC, Davtyan H, Thompson LM, Steffan JS, Svendsen CN, Blurton-Jones M. PMID: 28225193; PMCID: PMC5464354.
      View in: PubMed   Mentions: 9     Fields:    Translation:HumansAnimalsCells
    18. Serine 421 regulates mutant huntingtin toxicity and clearance in mice. J Clin Invest. 2016 09 01; 126(9):3585-97. Kratter IH, Zahed H, Lau A, Tsvetkov AS, Daub AC, Weiberth KF, Gu X, Saudou F, Humbert S, Yang XW, Osmand A, Steffan JS, Masliah E, Finkbeiner S. PMID: 27525439; PMCID: PMC5004962.
      View in: PubMed   Mentions: 28     Fields:    Translation:HumansAnimalsCells
    19. PIAS1 Regulates Mutant Huntingtin Accumulation and Huntington's Disease-Associated Phenotypes In Vivo. Neuron. 2016 05 04; 90(3):507-20. Ochaba J, Monteys AM, O'Rourke JG, Reidling JC, Steffan JS, Davidson BL, Thompson LM. PMID: 27146268; PMCID: PMC4942306.
      View in: PubMed   Mentions: 46     Fields:    Translation:HumansAnimalsCells
    20. A cause for childhood ataxia. Elife. 2016 Mar 01; 5. Steffan JS. PMID: 26929993; PMCID: PMC4786425.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    21. Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy. 2016; 12(1):1-222. Klionsky DJ, Abdelmohsen K, Abe A, Abedin MJ, Abeliovich H, Acevedo Arozena A, Adachi H, Adams CM, Adams PD, Adeli K, Adhihetty PJ, Adler SG, Agam G, Agarwal R, Aghi MK, Agnello M, Agostinis P, Aguilar PV, Aguirre-Ghiso J, Airoldi EM, Ait-Si-Ali S, Akematsu T, Akporiaye ET, Al-Rubeai M, Albaiceta GM, Albanese C, Albani D, Albert ML, Aldudo J, Algül H, Alirezaei M, Alloza I, Almasan A, Almonte-Beceril M, Alnemri ES, Alonso C, Altan-Bonnet N, Altieri DC, Alvarez S, Alvarez-Erviti L, Alves S, Amadoro G, Amano A, Amantini C, Ambrosio S, Amelio I, Amer AO, Amessou M, Amon A, An Z, Anania FA, Andersen SU, Andley UP, Andreadi CK, Andrieu-Abadie N, Anel A, Ann DK, Anoopkumar-Dukie S, Antonioli M, Aoki H, Apostolova N, Aquila S, Aquilano K, Araki K, Arama E, Aranda A, Araya J, Arcaro A, Arias E, Arimoto H, Ariosa AR, Armstrong JL, Arnould T, Arsov I, Asanuma K, Askanas V, Asselin E, Atarashi R, Atherton SS, Atkin JD, Attardi LD, Auberger P, Auburger G, Aurelian L, Autelli R, Avagliano L, Avantaggiati ML, Avrahami L, Awale S, Azad N, Bachetti T, Backer JM, Bae DH, Bae JS, Bae ON, Bae SH, Baehrecke EH, Baek SH, Baghdiguian S, Bagniewska-Zadworna A, Bai H, Bai J, Bai XY, Bailly Y, Balaji KN, Balduini W, Ballabio A, Balzan R, Banerjee R, Bánhegyi G, Bao H, Barbeau B, Barrachina MD, Barreiro E, Bartel B, Bartolomé A, Bassham DC, Bassi MT, Bast RC, Basu A, Batista MT, Batoko H, Battino M, Bauckman K, Baumgarner BL, Bayer KU, Beale R, Beaulieu JF, Beck GR, Becker C, Beckham JD, Bédard PA, Bednarski PJ, Begley TJ, Behl C, Behrends C, Behrens GM, Behrns KE, Bejarano E, Belaid A, Belleudi F, Bénard G, Berchem G, Bergamaschi D, Bergami M, Berkhout B, Berliocchi L, Bernard A, Bernard M, Bernassola F, Bertolotti A, Bess AS, Besteiro S, Bettuzzi S, Bhalla S, Bhattacharyya S, Bhutia SK, Biagosch C, Bianchi MW, Biard-Piechaczyk M, Billes V, Bincoletto C, Bingol B, Bird SW, Bitoun M, Bjedov I, Blackstone C, Blanc L, Blanco GA, Blomhoff HK, Boada-Romero E, Böckler S, Boes M, Boesze-Battaglia K, Boise LH, Bolino A, Boman A, Bonaldo P, Bordi M, Bosch J, Botana LM, Botti J, Bou G, Bouché M, Bouchecareilh M, Boucher MJ, Boulton ME, Bouret SG, Boya P, Boyer-Guittaut M, Bozhkov PV, Brady N, Braga VM, Brancolini C, Braus GH, Bravo-San Pedro JM, Brennan LA, Bresnick EH, Brest P, Bridges D, Bringer MA, Brini M, Brito GC, Brodin B, Brookes PS, Brown EJ, Brown K, Broxmeyer HE, Bruhat A, Brum PC, Brumell JH, Brunetti-Pierri N, Bryson-Richardson RJ, Buch S, Buchan AM, Budak H, Bulavin DV, Bultman SJ, Bultynck G, Bumbasirevic V, Burelle Y, Burke RE, Burmeister M, Bütikofer P, Caberlotto L, Cadwell K, Cahova M, Cai D, Cai J, Cai Q, Calatayud S, Camougrand N, Campanella M, Campbell GR, Campbell M, Campello S, Candau R, Caniggia I, Cantoni L, Cao L, Caplan AB, Caraglia M, Cardinali C, Cardoso SM, Carew JS, Carleton LA, Carlin CR, Carloni S, Carlsson SR, Carmona-Gutierrez D, Carneiro LA, Carnevali O, Carra S, Carrier A, Carroll B, Casas C, Casas J, Cassinelli G, Castets P, Castro-Obregon S, Cavallini G, Ceccherini I, Cecconi F, Cederbaum AI, Ceña V, Cenci S, Cerella C, Cervia D, Cetrullo S, Chaachouay H, Chae HJ, Chagin AS, Chai CY, Chakrabarti G, Chamilos G, Chan EY, Chan MT, Chandra D, Chandra P, Chang CP, Chang RC, Chang TY, Chatham JC, Chatterjee S, Chauhan S, Che Y, Cheetham ME, Cheluvappa R, Chen CJ, Chen G, Chen GC, Chen G, Chen H, Chen JW, Chen JK, Chen M, Chen M, Chen P, Chen Q, Chen Q, Chen SD, Chen S, Chen SS, Chen W, Chen WJ, Chen WQ, Chen W, Chen X, Chen YH, Chen YG, Chen Y, Chen Y, Chen Y, Chen YJ, Chen YQ, Chen Y, Chen Z, Chen Z, Cheng A, Cheng CH, Cheng H, Cheong H, Cherry S, Chesney J, Cheung CH, Chevet E, Chi HC, Chi SG, Chiacchiera F, Chiang HL, Chiarelli R, Chiariello M, Chieppa M, Chin LS, Chiong M, Chiu GN, Cho DH, Cho SG, Cho WC, Cho YY, Cho YS, Choi AM, et al. PMID: 26799652; PMCID: PMC4835977.
      View in: PubMed   Mentions: 2804     Fields:    Translation:HumansAnimals
    22. Treating the whole body in Huntington's disease. Lancet Neurol. 2015 Nov; 14(11):1135-42. Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ. PMID: 26466780.
      View in: PubMed   Mentions: 69     Fields:    Translation:Humans
    23. Potential function for the Huntingtin protein as a scaffold for selective autophagy. Proc Natl Acad Sci U S A. 2014 Nov 25; 111(47):16889-94. Ochaba J, Lukacsovich T, Csikos G, Zheng S, Margulis J, Salazar L, Mao K, Lau AL, Yeung SY, Humbert S, Saudou F, Klionsky DJ, Finkbeiner S, Zeitlin SO, Marsh JL, Housman DE, Thompson LM, Steffan JS. PMID: 25385587; PMCID: PMC4250109.
      View in: PubMed   Mentions: 146     Fields:    Translation:Animals
    24. Autophagy researchers. Autophagy. 2014 Feb; 10(2):188-91. Carra S, Malagoli D, Ney PA, Steffan JS. PMID: 24389674; PMCID: PMC5396088.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    25. SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Rep. 2013 Jul 25; 4(2):362-75. O'Rourke JG, Gareau JR, Ochaba J, Song W, Raskó T, Reverter D, Lee J, Monteys AM, Pallos J, Mee L, Vashishtha M, Apostol BL, Nicholson TP, Illes K, Zhu YZ, Dasso M, Bates GP, Difiglia M, Davidson B, Wanker EE, Marsh JL, Lima CD, Steffan JS, Thompson LM. PMID: 23871671; PMCID: PMC3931302.
      View in: PubMed   Mentions: 61     Fields:    Translation:HumansAnimalsCells
    26. Selective histone deacetylase (HDAC) inhibition imparts beneficial effects in Huntington's disease mice: implications for the ubiquitin-proteasomal and autophagy systems. Hum Mol Genet. 2012 Dec 15; 21(24):5280-93. Jia H, Kast RJ, Steffan JS, Thomas EA. PMID: 22965876; PMCID: PMC3510756.
      View in: PubMed   Mentions: 61     Fields:    Translation:AnimalsCells
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    28. Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice. Proc Natl Acad Sci U S A. 2012 Feb 28; 109(9):3528-33. Di Pardo A, Maglione V, Alpaugh M, Horkey M, Atwal RS, Sassone J, Ciammola A, Steffan JS, Fouad K, Truant R, Sipione S. PMID: 22331905; PMCID: PMC3295265.
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    31. Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron. 2009 Dec 24; 64(6):828-40. Gu X, Greiner ER, Mishra R, Kodali R, Osmand A, Finkbeiner S, Steffan JS, Thompson LM, Wetzel R, Yang XW. PMID: 20064390; PMCID: PMC2807408.
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    39. Targeting aggregation in the development of therapeutics for the treatment of Huntington's disease and other polyglutamine repeat diseases. Expert Opin Ther Targets. 2003 Apr; 7(2):201-13. Steffan JS, Thompson LM. PMID: 12667098.
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    40. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc Natl Acad Sci U S A. 2003 Feb 18; 100(4):2041-6. Hockly E, Richon VM, Woodman B, Smith DL, Zhou X, Rosa E, Sathasivam K, Ghazi-Noori S, Mahal A, Lowden PA, Steffan JS, Marsh JL, Thompson LM, Lewis CM, Marks PA, Bates GP. PMID: 12576549; PMCID: PMC149955.
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    41. A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila. Nat Genet. 2002 Apr; 30(4):367-76. Kazantsev A, Walker HA, Slepko N, Bear JE, Preisinger E, Steffan JS, Zhu YZ, Gertler FB, Housman DE, Marsh JL, Thompson LM. PMID: 11925563.
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