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Dysregulation of protein SUMOylation networks in Huntington's disease R6/2 mouse striatum. Brain. 2025 Apr 03; 148(4):1212-1227.
Kachemov M, Vaibhav V, Smith C, Sundararaman N, Heath M, Pendlebury DF, Matlock A, Lau A, Morozko E, Lim RG, Reidling J, Steffan JS, Van Eyk JE, Thompson LM. PMID: 39391934; PMCID: PMC11969464.
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1 Fields:
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AnimalsCells
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Aberrant splicing in Huntington's disease accompanies disrupted TDP-43 activity and altered m6A RNA modification. Nat Neurosci. 2025 Feb; 28(2):280-292.
Nguyen TB, Miramontes R, Chillon-Marinas C, Maimon R, Vazquez-Sanchez S, Lau AL, McClure NR, Wu Z, Wang KQ, England WE, Singha M, Stocksdale JT, Heath M, Jang KH, Jung S, Ling K, Jafar-Nejad P, McKnight JI, Ho LN, Dalahmah OA, Faull RLM, Steffan JS, Reidling JC, Jang C, Lee G, Cleveland DW, Lagier-Tourenne C, Spitale RC, Thompson LM. PMID: 39762660; PMCID: PMC11802453.
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PubMed Mentions:
2 Fields:
Translation:
HumansAnimalsCells
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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; PMCID: PMC11317567.
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PubMed Mentions:
3 Fields:
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HumansAnimalsCells
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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.
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2
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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.
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APOE4 dysregulates autophagy in cultured cells. Autophagy Rep. 2022; 1(1):29-33.
Fote GM, Steffan JS. PMID: 38912292; PMCID: PMC11192451.
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1
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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.
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PubMed Mentions:
15 Fields:
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AnimalsCells
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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.
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PubMed Mentions:
5 Fields:
Translation:
AnimalsCells
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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.
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PubMed Mentions:
10
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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.
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PubMed Mentions:
1 Fields:
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AnimalsCells
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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.
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1158 Fields:
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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.
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26 Fields:
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HumansAnimalsCells
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A Scoping Review of Dietary Factors Conferring Risk or Protection for Cognitive Decline in APOE ε4 Carriers. J Nutr Health Aging. 2021; 25(10):1167-1178.
Fote GM, Geller NR, Reyes-Ortiz AM, Thompson LM, Steffan JS, Grill JD. PMID: 34866144; PMCID: PMC8890439.
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PubMed Mentions:
2 Fields:
Translation:
Humans
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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.
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PubMed Mentions:
124 Fields:
Translation:
AnimalsCells
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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.
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PubMed Mentions:
3 Fields:
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HumansAnimalsCells
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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.
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PubMed Mentions:
18 Fields:
Translation:
AnimalsCells
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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.
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PubMed Mentions:
10 Fields:
Translation:
Animals
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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.
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PubMed Mentions:
3 Fields:
Translation:
HumansAnimals
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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.
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PubMed Mentions:
41 Fields:
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HumansAnimalsCells
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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.
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PubMed Mentions:
10 Fields:
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HumansAnimalsCells
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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.
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PubMed Mentions:
27 Fields:
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HumansAnimalsCells
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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.
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PubMed Mentions:
41 Fields:
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HumansAnimalsCells
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A cause for childhood ataxia. Elife. 2016 Mar 01; 5.
Steffan JS. PMID: 26929993; PMCID: PMC4786425.
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Humans
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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.
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2748 Fields:
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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.
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66 Fields:
Translation:
Humans
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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.
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PubMed Mentions:
137 Fields:
Translation:
Animals
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Autophagy researchers. Autophagy. 2014 Feb; 10(2):188-91.
Carra S, Malagoli D, Ney PA, Steffan JS. PMID: 24389674; PMCID: PMC5396088.
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Humans
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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.
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PubMed Mentions:
65 Fields:
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HumansAnimalsCells
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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.
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PubMed Mentions:
62 Fields:
Translation:
AnimalsCells
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Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy. 2012 Apr; 8(4):445-544.
Klionsky DJ, Abdalla FC, Abeliovich H, Abraham RT, Acevedo-Arozena A, Adeli K, Agholme L, Agnello M, Agostinis P, Aguirre-Ghiso JA, Ahn HJ, Ait-Mohamed O, Ait-Si-Ali S, Akematsu T, Akira S, Al-Younes HM, Al-Zeer MA, Albert ML, Albin RL, Alegre-Abarrategui J, Aleo MF, Alirezaei M, Almasan A, Almonte-Becerril M, Amano A, Amaravadi R, Amarnath S, Amer AO, Andrieu-Abadie N, Anantharam V, Ann DK, Anoopkumar-Dukie S, Aoki H, Apostolova N, Arancia G, Aris JP, Asanuma K, Asare NY, Ashida H, Askanas V, Askew DS, Auberger P, Baba M, Backues SK, Baehrecke EH, Bahr BA, Bai XY, Bailly Y, Baiocchi R, Baldini G, Balduini W, Ballabio A, Bamber BA, Bampton ET, Bánhegyi G, Bartholomew CR, Bassham DC, Bast RC, Batoko H, Bay BH, Beau I, Béchet DM, Begley TJ, Behl C, Behrends C, Bekri S, Bellaire B, Bendall LJ, Benetti L, Berliocchi L, Bernardi H, Bernassola F, Besteiro S, Bhatia-Kissova I, Bi X, Biard-Piechaczyk M, Blum JS, Boise LH, Bonaldo P, Boone DL, Bornhauser BC, Bortoluci KR, Bossis I, Bost F, Bourquin JP, Boya P, Boyer-Guittaut M, Bozhkov PV, Brady NR, Brancolini C, Brech A, Brenman JE, Brennand A, Bresnick EH, Brest P, Bridges D, Bristol ML, Brookes PS, Brown EJ, Brumell JH, Brunetti-Pierri N, Brunk UT, Bulman DE, Bultman SJ, Bultynck G, Burbulla LF, Bursch W, Butchar JP, Buzgariu W, Bydlowski SP, Cadwell K, Cahová M, Cai D, Cai J, Cai Q, Calabretta B, Calvo-Garrido J, Camougrand N, Campanella M, Campos-Salinas J, Candi E, Cao L, Caplan AB, Carding SR, Cardoso SM, Carew JS, Carlin CR, Carmignac V, Carneiro LA, Carra S, Caruso RA, Casari G, Casas C, Castino R, Cebollero E, Cecconi F, Celli J, Chaachouay H, Chae HJ, Chai CY, Chan DC, Chan EY, Chang RC, Che CM, Chen CC, Chen GC, Chen GQ, Chen M, Chen Q, Chen SS, Chen W, Chen X, Chen X, Chen X, Chen YG, Chen Y, Chen Y, Chen YJ, Chen Z, Cheng A, Cheng CH, Cheng Y, Cheong H, Cheong JH, Cherry S, Chess-Williams R, Cheung ZH, Chevet E, Chiang HL, Chiarelli R, Chiba T, Chin LS, Chiou SH, Chisari FV, Cho CH, Cho DH, Choi AM, Choi D, Choi KS, Choi ME, Chouaib S, Choubey D, Choubey V, Chu CT, Chuang TH, Chueh SH, Chun T, Chwae YJ, Chye ML, Ciarcia R, Ciriolo MR, Clague MJ, Clark RS, Clarke PG, Clarke R, Codogno P, Coller HA, Colombo MI, Comincini S, Condello M, Condorelli F, Cookson MR, Coombs GH, Coppens I, Corbalan R, Cossart P, Costelli P, Costes S, Coto-Montes A, Couve E, Coxon FP, Cregg JM, Crespo JL, Cronjé MJ, Cuervo AM, Cullen JJ, Czaja MJ, D'Amelio M, Darfeuille-Michaud A, Davids LM, Davies FE, De Felici M, de Groot JF, de Haan CA, De Martino L, De Milito A, De Tata V, Debnath J, Degterev A, Dehay B, Delbridge LM, Demarchi F, Deng YZ, Dengjel J, Dent P, Denton D, Deretic V, Desai SD, Devenish RJ, Di Gioacchino M, Di Paolo G, Di Pietro C, Díaz-Araya G, Díaz-Laviada I, Diaz-Meco MT, Diaz-Nido J, Dikic I, Dinesh-Kumar SP, Ding WX, Distelhorst CW, Diwan A, Djavaheri-Mergny M, Dokudovskaya S, Dong Z, Dorsey FC, Dosenko V, Dowling JJ, Doxsey S, Dreux M, Drew ME, Duan Q, Duchosal MA, Duff K, Dugail I, Durbeej M, Duszenko M, Edelstein CL, Edinger AL, Egea G, Eichinger L, Eissa NT, Ekmekcioglu S, El-Deiry WS, Elazar Z, Elgendy M, Ellerby LM, Eng KE, Engelbrecht AM, Engelender S, Erenpreisa J, Escalante R, Esclatine A, Eskelinen EL, Espert L, Espina V, Fan H, Fan J, Fan QW, Fan Z, Fang S, Fang Y, Fanto M, Fanzani A, Farkas T, Farré JC, Faure M, Fechheimer M, Feng CG, Feng J, Feng Q, Feng Y, Fésüs L, Feuer R, Figueiredo-Pereira ME, Fimia GM, Fingar DC, Finkbeiner S, Finkel T, Finley KD, Fiorito F, Fisher EA, Fisher PB, Flajolet M, Florez-McClure ML, Florio S, Fon EA, Fornai F, Fortunato F, Fotedar R, Fowler DH, Fox HS, Franco R, Frankel LB, Fransen M, Fuentes JM, Fueyo J, Fujii J, Fujisaki K, et al. PMID: 22966490; PMCID: PMC3404883.
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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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79 Fields:
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AnimalsCells
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Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice. J Huntingtons Dis. 2012; 1(1):71-87.
Neveklovska M, Clabough EB, Steffan JS, Zeitlin SO. PMID: 22956985; PMCID: PMC3432989.
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12 Fields:
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AnimalsCells
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Does Huntingtin play a role in selective macroautophagy? Cell Cycle. 2010 Sep 01; 9(17):3401-13.
Steffan JS. PMID: 20703094; PMCID: PMC3047613.
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46 Fields:
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HumansAnimalsCells
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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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163 Fields:
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IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J Cell Biol. 2009 Dec 28; 187(7):1083-99.
Thompson LM, Aiken CT, Kaltenbach LS, Agrawal N, Illes K, Khoshnan A, Martinez-Vincente M, Arrasate M, O'Rourke JG, Khashwji H, Lukacsovich T, Zhu YZ, Lau AL, Massey A, Hayden MR, Zeitlin SO, Finkbeiner S, Green KN, LaFerla FM, Bates G, Huang L, Patterson PH, Lo DC, Cuervo AM, Marsh JL, Steffan JS. PMID: 20026656; PMCID: PMC2806289.
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179 Fields:
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Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity. J Biol Chem. 2009 Oct 23; 284(43):29427-36.
Aiken CT, Steffan JS, Guerrero CM, Khashwji H, Lukacsovich T, Simmons D, Purcell JM, Menhaji K, Zhu YZ, Green K, Laferla F, Huang L, Thompson LM, Marsh JL. PMID: 19710014; PMCID: PMC2785575.
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78 Fields:
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HumansCells
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Nicotinamide restores cognition in Alzheimer's disease transgenic mice via a mechanism involving sirtuin inhibition and selective reduction of Thr231-phosphotau. J Neurosci. 2008 Nov 05; 28(45):11500-10.
Green KN, Steffan JS, Martinez-Coria H, Sun X, Schreiber SS, Thompson LM, LaFerla FM. PMID: 18987186; PMCID: PMC2617713.
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150 Fields:
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Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease. Hum Mol Genet. 2008 Dec 01; 17(23):3767-75.
Pallos J, Bodai L, Lukacsovich T, Purcell JM, Steffan JS, Thompson LM, Marsh JL. PMID: 18762557; PMCID: PMC2581431.
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115 Fields:
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HumansAnimalsCells
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The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. Hum Mol Genet. 2007 Jan 01; 16(1):61-77.
Rockabrand E, Slepko N, Pantalone A, Nukala VN, Kazantsev A, Marsh JL, Sullivan PG, Steffan JS, Sensi SL, Thompson LM. PMID: 17135277.
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109 Fields:
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AnimalsCells
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Normal-repeat-length polyglutamine peptides accelerate aggregation nucleation and cytotoxicity of expanded polyglutamine proteins. Proc Natl Acad Sci U S A. 2006 Sep 26; 103(39):14367-72.
Slepko N, Bhattacharyya AM, Jackson GR, Steffan JS, Marsh JL, Thompson LM, Wetzel R. PMID: 16980414; PMCID: PMC1599969.
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20 Fields:
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HumansAnimalsCells
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SUMO modification of Huntingtin and Huntington's disease pathology. Science. 2004 Apr 02; 304(5667):100-4.
Steffan JS, Agrawal N, Pallos J, Rockabrand E, Trotman LC, Slepko N, Illes K, Lukacsovich T, Zhu YZ, Cattaneo E, Pandolfi PP, Thompson LM, Marsh JL. PMID: 15064418.
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259 Fields:
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HumansAnimalsCells
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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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3 Fields:
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HumansAnimalsCells
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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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262 Fields:
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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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44 Fields:
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AnimalsCells
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Histone deacetylase inhibitors reduce polyglutamine toxicity. Proc Natl Acad Sci U S A. 2001 Dec 18; 98(26):15179-84.
McCampbell A, Taye AA, Whitty L, Penney E, Steffan JS, Fischbeck KH. PMID: 11742087; PMCID: PMC65003.
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54 Fields:
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AnimalsCells
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Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature. 2001 Oct 18; 413(6857):739-43.
Steffan JS, Bodai L, Pallos J, Poelman M, McCampbell A, Apostol BL, Kazantsev A, Schmidt E, Zhu YZ, Greenwald M, Kurokawa R, Housman DE, Jackson GR, Marsh JL, Thompson LM. PMID: 11607033.
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361 Fields:
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AnimalsCells
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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A. 2000 Jun 06; 97(12):6763-8.
Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM. PMID: 10823891; PMCID: PMC18731.
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329 Fields:
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HumansAnimalsCells
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Interaction of TATA-binding protein with upstream activation factor is required for activated transcription of ribosomal DNA by RNA polymerase I in Saccharomyces cerevisiae in vivo. Mol Cell Biol. 1998 Jul; 18(7):3752-61.
Steffan JS, Keys DA, Vu L, Nomura M. PMID: 9632758; PMCID: PMC108958.
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22 Fields:
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AnimalsCells
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The role of TBP in rDNA transcription by RNA polymerase I in Saccharomyces cerevisiae: TBP is required for upstream activation factor-dependent recruitment of core factor. Genes Dev. 1996 Oct 15; 10(20):2551-63.
Steffan JS, Keys DA, Dodd JA, Nomura M. PMID: 8895657.
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52 Fields:
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AnimalsCells
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RRN11 encodes the third subunit of the complex containing Rrn6p and Rrn7p that is essential for the initiation of rDNA transcription by yeast RNA polymerase I. J Biol Chem. 1996 Aug 30; 271(35):21062-7.
Lalo D, Steffan JS, Dodd JA, Nomura M. PMID: 8702872.
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42 Fields:
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HumansAnimalsCells
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Expression and function of a mislocalized form of peroxisomal malate dehydrogenase (MDH3) in yeast. J Biol Chem. 1995 Sep 08; 270(36):21220-5.
McAlister-Henn L, Steffan JS, Minard KI, Anderson SL. PMID: 7673155.
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13 Fields:
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AnimalsCells
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RRN6 and RRN7 encode subunits of a multiprotein complex essential for the initiation of rDNA transcription by RNA polymerase I in Saccharomyces cerevisiae. Genes Dev. 1994 Oct 01; 8(19):2349-62.
Keys DA, Vu L, Steffan JS, Dodd JA, Yamamoto RT, Nogi Y, Nomura M. PMID: 7958901.
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44 Fields:
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AnimalsCells
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Isolation and characterization of the yeast gene encoding the MDH3 isozyme of malate dehydrogenase. J Biol Chem. 1992 Dec 05; 267(34):24708-15.
Steffan JS, McAlister-Henn L. PMID: 1447211.
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28 Fields:
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AnimalsCells
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Expression and function of heterologous forms of malate dehydrogenase in yeast. Arch Biochem Biophys. 1992 Feb 14; 293(1):93-102.
Steffan JS, Minard KI, McAlister-Henn L. PMID: 1731644.
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1 Fields:
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AnimalsCells
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Structural and functional effects of mutations altering the subunit interface of mitochondrial malate dehydrogenase. Arch Biochem Biophys. 1991 Jun; 287(2):276-82.
Steffan JS, McAlister-Henn L. PMID: 1898005.
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6 Fields:
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AnimalsCells
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Gene sequence and primary structure of mitochondrial malate dehydrogenase from Saccharomyces cerevisiae. Biochemistry. 1988 Nov 01; 27(22):8393-400.
Thompson LM, Sutherland P, Steffan JS, McAlister-Henn L. PMID: 3072021.
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10 Fields:
Translation:
AnimalsCells