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JUAN BOTAS

TitleProfessor
InstitutionBaylor College of Medicine
DepartmentDepartment of Molecular & Human Genetics
Address1250 MOURSUND STREET
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    U01AG072439     (SHULMAN, JOSHUA M)Jul 1, 2021 - Jun 30, 2026
    NIH
    Functional Genomic Dissection of Alzheimer's Disease in Humans and Drosophila Models
    Role: Co-Principal Investigator
    R01AG057339     (BOTAS, JUAN)Sep 15, 2017 - May 31, 2022
    NIH
    Functional Dissection of Alzheimer's Disease Networks in Drosophila: from Association to Causal Modulators of Age-Dependent Neurodegeration
    Role: Principal Investigator
    R01NS042179     (BOTAS, JUAN)Jul 1, 2001 - Jun 30, 2015
    NIH
    Probing Neurodegeneration with Drosophila
    Role: Principal Investigator
    R56NS042179     (BOTAS, JUAN)Jul 1, 2001 - Jun 30, 2009
    NIH
    Probing Neurodegeneration with Drosophila
    Role: Principal Investigator
    R01GM055681     (BOTAS, JUAN)Sep 24, 1997 - Mar 31, 2006
    NIH
    A genetic analysis of Drosophila limb patterning
    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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    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. Manor J, Jangam SV, Chung HL, Bhagwat P, Andrews J, Chester H, Kondo S, Srivastav S, Botas J, Moser AB, Huguenin SM, Wangler MF. Genetic analysis of the X-linked Adrenoleukodystrophy ABCD1 gene in Drosophila uncovers a role in Peroxisomal dynamics. bioRxiv. 2024 Sep 25. PMID: 39386423; PMCID: PMC11463603.
      Citations:    
    2. Sonn JY, Kim W, Iwanaszko M, Aoi Y, Li Y, Parkitny L, Brissette JL, Weiner L, Al-Ramahi I, Botas J, Shilatifard A, Zoghbi HY. MeCP2 Interacts with the Super Elongation Complex to Regulate Transcription. bioRxiv. 2024 Jul 01. PMID: 39005382; PMCID: PMC11244860.
      Citations:    
    3. Han X, Wang W, Ma LH, Ai-Ramahi I, Botas J, MacKenzie K, Allen GI, Young DW, Liu Z, Maletic-Savatic M. SPA-STOCSY: an automated tool for identifying annotated and non-annotated metabolites in high-throughput NMR spectra. Bioinformatics. 2023 10 03; 39(10). PMID: 37792497; PMCID: PMC10568371.
      Citations:    Fields:    Translation:HumansAnimals
    4. Wu T, Deger JM, Ye H, Guo C, Dhindsa J, Pekarek BT, Al-Ouran R, Liu Z, Al-Ramahi I, Botas J, Shulman JM. Tau polarizes an aging transcriptional signature to excitatory neurons and glia. Elife. 2023 May 23; 12. PMID: 37219079; PMCID: PMC10259480.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    5. Yu M, Ye H, De-Paula RB, Mangleburg CG, Wu T, Lee TV, Li Y, Duong D, Phillips B, Cruchaga C, Allen GI, Seyfried NT, Al-Ramahi I, Botas J, Shulman JM. Functional screening of lysosomal storage disorder genes identifies modifiers of alpha-synuclein neurotoxicity. PLoS Genet. 2023 05; 19(5):e1010760. PMID: 37200393; PMCID: PMC10231792.
      Citations: 1     Fields:    Translation:HumansAnimalsCells
    6. Bourquard T, Lee K, Al-Ramahi I, Pham M, Shapiro D, Lagisetty Y, Soleimani S, Mota S, Wilhelm K, Samieinasab M, Kim YW, Huh E, Asmussen J, Katsonis P, Botas J, Lichtarge O. Functional variants identify sex-specific genes and pathways in Alzheimer's Disease. Nat Commun. 2023 05 13; 14(1):2765. PMID: 37179358; PMCID: PMC10183026.
      Citations: 4     Fields:    Translation:Humans
    7. Hernandez SJ, Lim RG, Onur T, Dane MA, Smith R, Wang K, Jean GE, Reyes-Ortiz A, Devlin K, Miramontes R, Wu J, Casale M, Kilburn D, Heiser LM, Korkola JE, Van Vactor D, Botas J, Thompson-Peer KL, Thompson LM. An altered extracellular matrix-integrin interface contributes to Huntington's disease-associated CNS dysfunction in glial and vascular cells. Hum Mol Genet. 2023 04 20; 32(9):1483-1496. PMID: 36547263; PMCID: PMC10117161.
      Citations:    Fields:    Translation:HumansCells
    8. Han X, Wang W, Ma LH, Al-Ramahi I, Botas J, MacKenzie K, Allen GI, Young DW, Liu Z, Maletic-Savatic M. SPA-STOCSY: An Automated Tool for Identification of Annotated and Non-Annotated Metabolites in High-Throughput NMR Spectra. bioRxiv. 2023 Feb 22. PMID: 36865102; PMCID: PMC9980041.
      Citations:    
    9. Li J, Amoh BK, McCormick E, Tarkunde A, Zhu KF, Perez A, Mair M, Moore J, Shulman JM, Al-Ramahi I, Botas J. Integration of transcriptome-wide association study with neuronal dysfunction assays provides functional genomics evidence for Parkinson's disease genes. Hum Mol Genet. 2023 01 27; 32(4):685-695. PMID: 36173927; PMCID: PMC9896475.
      Citations: 1     Fields:    Translation:AnimalsCells
    10. Kim J, de Haro M, Al-Ramahi I, Garaicoechea LL, Jeong HH, Sonn JY, Tadros B, Liu Z, Botas J, Zoghbi HY. Evolutionarily conserved regulators of tau identify targets for new therapies. Neuron. 2023 03 15; 111(6):824-838.e7. PMID: 36610398.
      Citations: 5     Fields:    Translation:HumansAnimals
    11. Zhang X, Wen X, Al-Ramahi I, Botas J, Lu B, Fu Y. Correction: Inhibition of HIPK3 by AST487 Ameliorates Mutant HTT-Induced Neurotoxicity and Apoptosis via Enhanced Autophagy. Neurosci Bull. 2022 Sep; 38(9):1123. PMID: 35864371; PMCID: PMC9468195.
      Citations:    Fields:    
    12. Lagisetty Y, Bourquard T, Al-Ramahi I, Mangleburg CG, Mota S, Soleimani S, Shulman JM, Botas J, Lee K, Lichtarge O. Identification of risk genes for Alzheimer's disease by gene embedding. Cell Genom. 2022 Sep 14; 2(9). PMID: 36268052; PMCID: PMC9581494.
      Citations: 2     
    13. Lee WS, Al-Ramahi I, Jeong HH, Jang Y, Lin T, Adamski CJ, Lavery LA, Rath S, Richman R, Bondar VV, Alcala E, Revelli JP, Orr HT, Liu Z, Botas J, Zoghbi HY. Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1. J Clin Invest. 2022 05 02; 132(9). PMID: 35499073; PMCID: PMC9057624.
      Citations:    Fields:    Translation:HumansAnimals
    14. Song H, Wang C, Zhu C, Wang Z, Yang H, Wu P, Cui X, Botas J, Dang Y, Ding Y, Fei Y, Lu B. Suppression of toxicity of the mutant huntingtin protein by its interacting compound, desonide. Proc Natl Acad Sci U S A. 2022 03 08; 119(10):e2114303119. PMID: 35238684; PMCID: PMC8917382.
      Citations:    Fields:    Translation:Animals
    15. Greco TM, Federspiel JD, Liu JP, Perez AM, Al-Ramahi I, Cantle JP, Carroll JB, Botas J, Zeitlin SO, Cristea IM, Secker C, Ramos ES, Wanker EE. Dynamics of huntingtin protein interactions in the striatum identifies candidate modifiers of Huntington disease. Cell Syst. 2022 04 20; 13(4):304-320.e5. PMID: 35148841; PMCID: PMC9317655.
      Citations:    Fields:    Translation:Animals
    16. Stephens MC, Brandt V, Botas J. The developmental roots of neurodegeneration. Neuron. 2022 01 05; 110(1):1-3. PMID: 34990574.
      Citations:    Fields:    Translation:HumansCells
    17. Zhang X, Wen X, Al-Ramahi I, Botas J, Lu B, Fu Y. Inhibition of HIPK3 by AST487 Ameliorates Mutant HTT-Induced Neurotoxicity and Apoptosis via Enhanced Autophagy. Neurosci Bull. 2022 Jan; 38(1):99-103. PMID: 34741261; PMCID: PMC8782961.
      Citations:    Fields:    Translation:Humans
    18. Suart CE, Perez AM, Al-Ramahi I, Maiuri T, Botas J, Truant R. Spinocerebellar Ataxia Type 1 protein Ataxin-1 is signaled to DNA damage by ataxia-telangiectasia mutated kinase. Hum Mol Genet. 2021 05 17; 30(8):706-715. PMID: 33772540.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    19. Onur TS, Laitman A, Zhao H, Keyho R, Kim H, Wang J, Mair M, Wang H, Li L, Perez A, de Haro M, Wan YW, Allen G, Lu B, Al-Ramahi I, Liu Z, Botas J. Downregulation of glial genes involved in synaptic function mitigates Huntington's disease pathogenesis. Elife. 2021 04 19; 10. PMID: 33871358; PMCID: PMC8149125.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    20. Lee WS, Lavery L, Rousseaux MWC, Rutledge EB, Jang Y, Wan YW, Wu SR, Kim W, Al-Ramahi I, Rath S, Adamski CJ, Bondar VV, Tewari A, Soleimani S, Mota S, Yalamanchili HK, Orr HT, Liu Z, Botas J, Zoghbi HY. Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1. EMBO J. 2021 04 01; 40(7):e106106. PMID: 33709453; PMCID: PMC8013850.
      Citations: 3     Fields:    Translation:HumansAnimalsCells
    21. Klionsky DJ, Abdel-Aziz AK, Abdelfatah S, Abdellatif M, Abdoli A, Abel S, Abeliovich H, Abildgaard MH, 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, Akoumianaki T, Akpinar HA, Al-Abd AM, Al-Akra L, Al-Gharaibeh A, Alaoui-Jamali MA, Alberti S, Alessandri C, Ali M, Alim Al-Bari MA, Aliwaini S, Alizadeh J, Almasan A, Alonso A, Altan-Bonnet N, Altieri DC, Alzaharna MM, Amadio M, Amantini C, Amaral C, Ambrosio S, Amer AO, Ammanathan V, An Z, Andersen SU, Andrabi SA, Andres AM, Angelini S, Ann D, Anozie UC, Ansari MY, Antebi A, Anwar T, Apetoh L, Apostolova N, Araki T, Araki Y, Arasaki K, Araya J, Arden C, Arguelles S, Arias E, Arikkath J, Arimoto H, Ariosa AR, Armstrong-James D, Aroca A, Arsov I, Asaro DML, Aschner M, Ashur-Fabian O, Atanasov AG, Au AK, Auberger P, Auner HW, Aurelian L, Autelli R, Avagliano L, 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, Bao Y, Baracca A, Barbati C, Bargiela A, 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, Baumgarner BL, Beale R, Bechara LRG, Beck GR, Beers MF, Begun J, Behrens GMN, Bei R, Bejarano E, Bel S, Behl C, Belaid A, Bellarosa C, Belleudi F, Benbrook DM, Bendorius M, Benitez BA, Benito-Cuesta I, Bensalem J, Berchtold MW, Berezowska S, Bergamaschi D, Bergami M, Bergmann A, Berliocchi 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, Birgisdottir AB, Bjorkoy G, Blanco G, Blas-Garcia A, Blasiak J, Blomgren K, Blum JS, Boada-Romero E, Boban M, Boesze-Battaglia K, Boeuf P, Boland B, Bomont P, Bonaldo P, Bonfili L, Bonifacino JS, Boone BA, Bootman MD, Bordi M, Borner C, Bornhauser BC, Borthakur G, Bose S, Botana LM, Botas J, Boulton ME, Bourdenx M, Bourgeois B, Bourke NM, Bousquet G, Bozhkov PV, Bozi LHM, Bozkurt TO, Brackney DE, Brandts CH, Braun RJ, 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, Bueno M, Buitrago-Molina LE, Buraschi S, Buch S, Buchan JR, Buckingham EM, Budak H, Bultynck G, Burada F, Burgoyne JR, Bustos V, Butturini E, Byrd A, Cadwell K, Cai J, Cai L, Cai Q, Calbet JA, Caldwell GA, Caldwell KA, Call JA, Calvani R, Camonis JH, Camougrand N, Campanella M, Campbell EM, Campello S, Campos JC, Canesi L, Caniggia I, Canonico B, Cao B, Caraglia M, Carchman EH, Cardenas C, Cardoso SM, Carew JS, Carleton G, Carloni S, Carmona-Gutierrez D, Carneiro LA, Carnevali O, Carosi JM, Carra S, Carrier L, Carroll B, Carter AB, Carvalho AN, Casas J, Cassioli C, Castillo EF, Castillo-Lluva S, Castori M, Castro-Caldas M, Catz SD, Cavadas C, Cavaliere F, Cavallini G, Cavinato M, Cayuela ML, Cebollada Rica P, Cecarini V, Cechowska-Pasko M, Cenci S, Cervia D, Cetintas VB, Cetrullo S, Chae HJ, Chagin AS, Chai CY, Chakrabarti G, Chakrabarti O, Chakraborty T, Chakraborty T, Chamilos G, Chan DW, Chan EYW, Chan ED, Chan HYE, Chan HH, Chan H, Chan MTV, Chan YS, Chandra PK, Chang CP, Chang C, Chang HC, Chang K, Chao J, Chapman T, Chatterjee S, Chaube SK, Chaudhary A, Chauhan S, Chaum E, Cheetham ME, Chen CS, Chen GC, Chen JF, Chen LL, Chen L, Chen L, Chen M, Chen MK, Chen N, Chen Q, Chen RH, et al. Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1. Autophagy. 2021 Jan; 17(1):1-382. PMID: 33634751; PMCID: PMC7996087.
      Citations: 357     Fields:    Translation:HumansAnimalsCells
    22. Kim YW, Al-Ramahi I, Koire A, Wilson SJ, Konecki DM, Mota S, Soleimani S, Botas J, Lichtarge O. Harnessing the paradoxical phenotypes of APOE ?2 and APOE ?4 to identify genetic modifiers in Alzheimer's disease. Alzheimers Dement. 2021 05; 17(5):831-846. PMID: 33576571; PMCID: PMC8247413.
      Citations: 7     Fields:    Translation:HumansAnimals
    23. Huichalaf CH, Al-Ramahi I, Park KW, Grunke SD, Lu N, de Haro M, El-Zein K, Gallego-Flores T, Perez AM, Jung SY, Botas J, Zoghbi HY, Jankowsky JL. Cross-species genetic screens to identify kinase targets for APP reduction in Alzheimer's disease. Hum Mol Genet. 2019 06 15; 28(12):2014-2029. PMID: 30753434; PMCID: PMC6548227.
      Citations: 1     Fields:    Translation:HumansAnimalsCells
    24. Rousseaux MWC, Al-Ramahi I, Jeong HH, Bajic A, Revelli JP, Ye H, Phan ET, Deger JM, Perez AM, Kim JY, Lavery LA, Xu Q, Li MZ, Kang H, Kim JJ, Shulman JM, Westbrook TF, Elledge SJ, Liu Z, Botas J, Zoghbi HY, V?zquez-V?lez GE. A Druggable Genome Screen Identifies Modifiers of a-Synuclein Levels via a Tiered Cross-Species Validation Approach. J Neurosci. 2018 10 24; 38(43):9286-9301. PMID: 30249792; PMCID: PMC6199406.
      Citations: 15     Fields:    Translation:HumansAnimalsCells
    25. Bondar VV, Adamski CJ, Onur TS, Tan Q, Wang L, Diaz-Garcia J, Park J, Orr HT, Botas J, Zoghbi HY. PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1. Hum Mol Genet. 2018 08 15; 27(16):2863-2873. PMID: 29860311; PMCID: PMC6077814.
      Citations: 7     Fields:    Translation:HumansAnimalsCells
    26. Al-Ramahi I, Lu B, Di Paola S, Pang K, de Haro M, Peluso I, Gallego-Flores T, Malik NT, Erikson K, Bleiberg BA, Avalos M, Fan G, Rivers LE, Laitman AM, Hild M, Palacino J, Liu Z, Medina DL, Botas J, Diaz-Garc?a JR. High-Throughput Functional Analysis Distinguishes Pathogenic, Nonpathogenic, and Compensatory Transcriptional Changes in Neurodegeneration. Cell Syst. 2018 07 25; 7(1):28-40.e4. PMID: 29936182; PMCID: PMC6082401.
      Citations: 13     Fields:    Translation:HumansAnimalsCells
    27. Al-Ramahi I, Giridharan SSP, Chen YC, Patnaik S, Safren N, Hasegawa J, de Haro M, Wagner Gee AK, Titus SA, Jeong H, Clarke J, Krainc D, Zheng W, Irvine RF, Barmada S, Ferrer M, Southall N, Weisman LS, Botas J, Marugan JJ. Inhibition of PIP4K? ameliorates the pathological effects of mutant huntingtin protein. Elife. 2017 12 26; 6. PMID: 29256861; PMCID: PMC5743427.
      Citations: 21     Fields:    Translation:HumansAnimalsCells
    28. Rousseaux MW, de Haro M, Lasagna-Reeves CA, De Maio A, Park J, Jafar-Nejad P, Al-Ramahi I, Sharma A, See L, Lu N, Vilanova-Velez L, Klisch TJ, Westbrook TF, Troncoso JC, Botas J, Zoghbi HY. TRIM28 regulates the nuclear accumulation and toxicity of both alpha-synuclein and tau. Elife. 2016 10 25; 5. PMID: 27779468; PMCID: PMC5104516.
      Citations: 34     Fields:    Translation:HumansAnimalsCells
    29. Lasagna-Reeves CA, de Haro M, Hao S, Park J, Rousseaux MW, Al-Ramahi I, Jafar-Nejad P, Vilanova-Velez L, See L, De Maio A, Nitschke L, Wu Z, Troncoso JC, Westbrook TF, Tang J, Botas J, Zoghbi HY. Reduction of Nuak1 Decreases Tau and Reverses Phenotypes in a Tauopathy Mouse Model. Neuron. 2016 Oct 19; 92(2):407-418. PMID: 27720485; PMCID: PMC5745060.
      Citations: 48     Fields:    Translation:HumansAnimalsCells
    30. Soriano S, Calap-Quintana P, Al-Ramahi I, Botas J, Llorens JV, Guti?rrez L, Mart?nez-Sebasti?n MJ, Molt? MD. Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease. PLoS One. 2016; 11(7):e0159209. PMID: 27433942; PMCID: PMC4951068.
      Citations: 14     Fields:    Translation:HumansAnimalsCells
    31. Chouhan AK, Guo C, Hsieh YC, Ye H, Senturk M, Zuo Z, Li Y, Chatterjee S, Botas J, Jackson GR, Bellen HJ, Shulman JM. Uncoupling neuronal death and dysfunction in Drosophila models of neurodegenerative disease. Acta Neuropathol Commun. 2016 06 23; 4(1):62. PMID: 27338814; PMCID: PMC4918017.
      Citations: 28     Fields:    Translation:HumansAnimalsCells
    32. Langfelder P, Cantle JP, Chatzopoulou D, Wang N, Gao F, Al-Ramahi I, Lu XH, Ramos EM, El-Zein K, Zhao Y, Deverasetty S, Tebbe A, Schaab C, Lavery DJ, Howland D, Kwak S, Botas J, Aaronson JS, Rosinski J, Coppola G, Horvath S, Yang XW. Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice. Nat Neurosci. 2016 Apr; 19(4):623-33. PMID: 26900923; PMCID: PMC5984042.
      Citations: 148     Fields:    Translation:Animals
    33. Espinosa AP, Leone L, Sardiello M, Ballabio A, Botas J, Bouch? V. Drosophila Mitf regulates the V-ATPase and the lysosomal-autophagic pathway. Autophagy. 2016; 12(3):484-98. PMID: 26761346; PMCID: PMC4835958.
      Citations: 48     Fields:    Translation:AnimalsCells
    34. Hangen E, Poizat G, Al-Ramahi I, Pardo R, Bourg N, Souquere S, Botas J, Humbert S, El-Daher MT, Bruy?re J, Mayet C, Pierron G, L?v?que-Fort S, Saudou F. Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. EMBO J. 2015 Sep 02; 34(17):2255-71. PMID: 26165689; PMCID: PMC4585462.
      Citations: 41     Fields:    Translation:HumansAnimalsCells
    35. Calap-Quintana P, Soriano S, Al-Ramahi I, Botas J, Llorens JV, Molt? MD, Mart?nez-Sebasti?n MJ. TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia. PLoS One. 2015; 10(7):e0132376. PMID: 26158631; PMCID: PMC4497667.
      Citations: 22     Fields:    Translation:HumansAnimals
    36. Yao Y, Cui X, Al-Ramahi I, Sun X, Li B, Hou J, Difiglia M, Palacino J, Wu ZY, Ma L, Botas J, Lu B. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. Elife. 2015 Mar 04; 4. PMID: 25738228; PMCID: PMC4372774.
      Citations: 34     Fields:    Translation:HumansAnimalsCells
    37. Lu XH, Mattis VB, Wang N, Al-Ramahi I, van den Berg N, Fratantoni SA, Waldvogel H, Greiner E, Osmand A, Elzein K, Xiao J, Dijkstra S, de Pril R, Vinters HV, Faull R, Signer E, Kwak S, Marugan JJ, Botas J, Fischer DF, Svendsen CN, Munoz-Sanjuan I, Yang XW. Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease. Sci Transl Med. 2014 Dec 24; 6(268):268ra178. PMID: 25540325.
      Citations: 57     Fields:    Translation:HumansAnimalsCells
    38. Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, Gao Y, Shaw CA, Arthur JSC, Orr HT, Westbrook TF, Botas J, Zoghbi HY. RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature. 2013 Jun 20; 498(7454):325-331. PMID: 23719381; PMCID: PMC4020154.
      Citations: 74     Fields:    Translation:HumansAnimalsCells
    39. de Haro M, Al-Ramahi I, Jones KR, Holth JK, Timchenko LT, Botas J. Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy. PLoS Genet. 2013 Apr; 9(4):e1003445. PMID: 23637619; PMCID: PMC3630084.
      Citations: 15     Fields:    Translation:HumansCells
    40. Lu B, Al-Ramahi I, Valencia A, Wang Q, Berenshteyn F, Yang H, Gallego-Flores T, Ichcho S, Lacoste A, Hild M, Difiglia M, Botas J, Palacino J. Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nat Neurosci. 2013 May; 16(5):562-70. PMID: 23525043.
      Citations: 47     Fields:    Translation:HumansAnimalsCells
    41. Holth JK, Bomben VC, Reed JG, Inoue T, Younkin L, Younkin SG, Pautler RG, Botas J, Noebels JL. Tau loss attenuates neuronal network hyperexcitability in mouse and Drosophila genetic models of epilepsy. J Neurosci. 2013 Jan 23; 33(4):1651-9. PMID: 23345237; PMCID: PMC3711605.
      Citations: 111     Fields:    Translation:Animals
    42. Miller JP, Yates BE, Al-Ramahi I, Berman AE, Sanhueza M, Kim E, de Haro M, DeGiacomo F, Torcassi C, Holcomb J, Gafni J, Mooney SD, Botas J, Ellerby LM, Hughes RE. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease. PLoS Genet. 2012; 8(11):e1003042. PMID: 23209424; PMCID: PMC3510027.
      Citations: 23     Fields:    Translation:HumansAnimalsCells
    43. Shirasaki DI, Greiner ER, Al-Ramahi I, Gray M, Boontheung P, Geschwind DH, Botas J, Coppola G, Horvath S, Loo JA, Yang XW. Network organization of the huntingtin proteomic interactome in mammalian brain. Neuron. 2012 Jul 12; 75(1):41-57. PMID: 22794259; PMCID: PMC3432264.
      Citations: 135     Fields:    Translation:HumansAnimals
    44. Zhang N, Li B, Al-Ramahi I, Cong X, Held JM, Kim E, Botas J, Gibson BW, Ellerby LM. Inhibition of lipid signaling enzyme diacylglycerol kinase epsilon attenuates mutant huntingtin toxicity. J Biol Chem. 2012 Jun 15; 287(25):21204-13. PMID: 22511757; PMCID: PMC3375542.
      Citations: 11     Fields:    Translation:HumansAnimalsCells
    45. Miller JP, Holcomb J, Al-Ramahi I, de Haro M, Gafni J, Zhang N, Kim E, Sanhueza M, Torcassi C, Kwak S, Botas J, Hughes RE, Ellerby LM. Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease. Neuron. 2010 Jul 29; 67(2):199-212. PMID: 20670829; PMCID: PMC3098887.
      Citations: 86     Fields:    Translation:HumansAnimalsCells
    46. Sofola O, Sundram V, Ng F, Kleyner Y, Morales J, Botas J, Jackson FR, Nelson DL. The Drosophila FMRP and LARK RNA-binding proteins function together to regulate eye development and circadian behavior. J Neurosci. 2008 Oct 08; 28(41):10200-5. PMID: 18842880; PMCID: PMC2587044.
      Citations: 24     Fields:    Translation:Animals
    47. Cukier HN, Perez AM, Collins AL, Zhou Z, Zoghbi HY, Botas J. Genetic modifiers of MeCP2 function in Drosophila. PLoS Genet. 2008 Sep 05; 4(9):e1000179. PMID: 18773074; PMCID: PMC2518867.
      Citations: 32     Fields:    Translation:HumansAnimalsCells
    48. Romero E, Cha GH, Verstreken P, Ly CV, Hughes RE, Bellen HJ, Botas J. Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm. Neuron. 2008 Jan 10; 57(1):27-40. PMID: 18184562; PMCID: PMC2277511.
      Citations: 77     Fields:    Translation:HumansAnimalsCells
    49. Al-Ramahi I, Lim J, Zhang M, Sorensen R, de Haro M, Branco J, Pulst SM, Zoghbi HY, Botas J, P?rez AM. dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet. 2007 Dec 28; 3(12):e234. PMID: 18166084; PMCID: PMC2323314.
      Citations: 53     Fields:    Translation:Animals
    50. Branco J, Al-Ramahi I, Ukani L, Fernandez-Funez P, Botas J, P?rez AM, Rinc?n-Limas D. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum Mol Genet. 2008 Feb 01; 17(3):376-90. PMID: 17984172.
      Citations: 43     Fields:    Translation:HumansAnimals
    51. Sofola OA, Jin P, Qin Y, Duan R, Liu H, de Haro M, Nelson DL, Botas J. RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS. Neuron. 2007 Aug 16; 55(4):565-71. PMID: 17698010; PMCID: PMC2215388.
      Citations: 189     Fields:    Translation:AnimalsCells
    52. Sofola OA, Jin P, Botas J, Nelson DL. Argonaute-2-dependent rescue of a Drosophila model of FXTAS by FRAXE premutation repeat. Hum Mol Genet. 2007 Oct 01; 16(19):2326-32. PMID: 17635840.
      Citations: 23     Fields:    Translation:HumansAnimalsCells
    53. Kaltenbach LS, Romero E, Becklin RR, Chettier R, Bell R, Phansalkar A, Strand A, Torcassi C, Savage J, Hurlburt A, Cha GH, Ukani L, Chepanoske CL, Zhen Y, Sahasrabudhe S, Olson J, Kurschner C, Ellerby LM, Peltier JM, Botas J, Hughes RE. Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet. 2007 May 11; 3(5):e82. PMID: 17500595; PMCID: PMC1866352.
      Citations: 183     Fields:    Translation:HumansAnimalsCells
    54. Botas J. Drosophila researchers focus on human disease. Nat Genet. 2007 May; 39(5):589-91. PMID: 17460691.
      Citations: 9     Fields:    Translation:HumansAnimals
    55. Qi ML, Tagawa K, Enokido Y, Yoshimura N, Wada Y, Watase K, Ishiura S, Kanazawa I, Botas J, Saitoe M, Wanker EE, Okazawa H. Proteome analysis of soluble nuclear proteins reveals that HMGB1/2 suppress genotoxic stress in polyglutamine diseases. Nat Cell Biol. 2007 Apr; 9(4):402-14. PMID: 17384639.
      Citations: 52     Fields:    Translation:AnimalsCells
    56. Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, Zoghbi HY. ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell. 2006 Dec 29; 127(7):1335-47. PMID: 17190598.
      Citations: 169     Fields:    Translation:HumansAnimalsCells
    57. Kadener S, Villella A, Kula E, Palm K, Pyza E, Botas J, Hall JC, Rosbash M. Neurotoxic protein expression reveals connections between the circadian clock and mating behavior in Drosophila. Proc Natl Acad Sci U S A. 2006 Sep 05; 103(36):13537-42. PMID: 16938865; PMCID: PMC1557390.
      Citations: 17     Fields:    Translation:AnimalsCells
    58. Al-Ramahi I, Lam YC, Chen HK, de Gouyon B, Zhang M, Branco J, de Haro M, Patterson C, Zoghbi HY, Botas J, P?rez AM. CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J Biol Chem. 2006 Sep 08; 281(36):26714-24. PMID: 16831871.
      Citations: 90     Fields:    Translation:HumansAnimalsCells
    59. Al-Ramahi I, De Gouyon B, Ukani L, Rosa A, Ashizawa T, Cooper TA, Botas J, de Haro M, Faustino NA. MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1. Hum Mol Genet. 2006 Jul 01; 15(13):2138-45. PMID: 16723374.
      Citations: 69     Fields:    Translation:HumansAnimalsCells
    60. Tsuda H, Jafar-Nejad H, Patel AJ, Sun Y, Chen HK, Rose MF, Venken KJ, Botas J, Orr HT, Bellen HJ, Zoghbi HY. The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins. Cell. 2005 Aug 26; 122(4):633-44. PMID: 16122429.
      Citations: 97     Fields:    Translation:AnimalsCells
    61. Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EM, Orr HT, Botas J, Zoghbi HY. Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1. Cell. 2003 May 16; 113(4):457-68. PMID: 12757707.
      Citations: 143     Fields:    Translation:HumansAnimalsCells
    62. Bergman CM, Pfeiffer BD, Hoskins RA, Gnirke A, Mungall CJ, Wang AM, Kronmiller B, Pacleb J, Park S, Stapleton M, Wan K, George RA, de Jong PJ, Botas J, Rubin GM, Celniker SE, Rinc?n-Limas DE. Assessing the impact of comparative genomic sequence data on the functional annotation of the Drosophila genome. Genome Biol. 2002; 3(12):RESEARCH0086. PMID: 12537575; PMCID: PMC151188.
      Citations: 69     Fields:    Translation:AnimalsCells
    63. Zoghbi HY, Botas J. Mouse and fly models of neurodegeneration. Trends Genet. 2002 Sep; 18(9):463-71. PMID: 12175807.
      Citations: 30     Fields:    Translation:HumansAnimals
    64. Kambris Z, Botas J, Capovilla M. Direct regulation of the muscle-identity gene apterous by a Hox protein in the somatic mesoderm. Development. 2001 Apr; 128(8):1221-30. PMID: 11262224.
      Citations: 26     Fields:    Translation:AnimalsCells
    65. Lu CH, Botas J, Rinc?n-Limas DE. Conserved overlapping and reciprocal expression of msh/Msx1 and apterous/Lhx2 in Drosophila and mice. Mech Dev. 2000 Dec; 99(1-2):177-81. PMID: 11091089.
      Citations: 4     Fields:    Translation:Animals
    66. Fernandez-Funez P, Nino-Rosales ML, de Gouyon B, She WC, Luchak JM, Martinez P, Turiegano E, Benito J, Capovilla M, Skinner PJ, McCall A, Canal I, Orr HT, Zoghbi HY, Botas J. Identification of genes that modify ataxin-1-induced neurodegeneration. Nature. 2000 Nov 02; 408(6808):101-6. PMID: 11081516.
      Citations: 234     Fields:    Translation:HumansAnimalsCells
    67. Lu CH, Canal I, Botas J, Rinc?n-Limas DE. The level of DLDB/CHIP controls the activity of the LIM homeodomain protein apterous: evidence for a functional tetramer complex in vivo. EMBO J. 2000 Jun 01; 19(11):2602-14. PMID: 10835358; PMCID: PMC212760.
      Citations: 15     Fields:    Translation:AnimalsCells
    68. Lilly B, O'Keefe DD, Thomas JB, Botas J. The LIM homeodomain protein dLim1 defines a subclass of neurons within the embryonic ventral nerve cord of Drosophila. Mech Dev. 1999 Nov; 88(2):195-205. PMID: 10534618.
      Citations: 12     Fields:    Translation:AnimalsCells
    69. Lu CH, Canal I, Calleja M, Botas J, Rinc?n-Limas DE, Rodr?guez-Esteban C, Izpis?a-Belmonte JC. Conservation of the expression and function of apterous orthologs in Drosophila and mammals. Proc Natl Acad Sci U S A. 1999 Mar 02; 96(5):2165-70. PMID: 10051612; PMCID: PMC26754.
      Citations: 33     Fields:    Translation:HumansAnimalsCells
    70. Lu CH, Botas J, Fern?ndez-F?nez P, Rinc?n-Limas DE, Garc?a-Bellido A. The relative expression amounts of apterous and its co-factor dLdb/Chip are critical for dorso-ventral compartmentalization in the Drosophila wing. EMBO J. 1998 Dec 01; 17(23):6846-53. PMID: 9843490; PMCID: PMC1171032.
      Citations: 20     Fields:    Translation:Animals
    71. Capovilla M, Botas J. Functional dominance among Hox genes: repression dominates activation in the regulation of Dpp. Development. 1998 Dec; 125(24):4949-57. PMID: 9811579.
      Citations: 16     Fields:    Translation:AnimalsCells
    72. Rodriguez-Esteban C, Schwabe JW, Rincon-Limas DE, Botas J, Pe?a JD, Magall?n J, Izpis?a Belmonte JC. Lhx2, a vertebrate homologue of apterous, regulates vertebrate limb outgrowth. Development. 1998 Oct; 125(20):3925-34. PMID: 9735354.
      Citations: 16     Fields:    Translation:AnimalsCells
    73. Bendall AJ, Botas J, Abate-Shen C, Rinc?n-Limas DE. Protein complex formation between Msx1 and Lhx2 homeoproteins is incompatible with DNA binding activity. Differentiation. 1998 Jul; 63(3):151-7. PMID: 9697309.
      Citations: 13     Fields:    Translation:AnimalsCells
    74. Botas J, Auwers L. Chromosomal binding sites of Ultrabithorax homeotic proteins. Mech Dev. 1996 May; 56(1-2):129-38. PMID: 8798153.
      Citations: 7     Fields:    Translation:AnimalsCells
    75. Chan SK, Jaffe L, Capovilla M, Botas J, Mann RS. The DNA binding specificity of Ultrabithorax is modulated by cooperative interactions with extradenticle, another homeoprotein. Cell. 1994 Aug 26; 78(4):603-15. PMID: 7915199.
      Citations: 132     Fields:    Translation:AnimalsCells
    76. Eldon E, Kooyer S, D'Evelyn D, Duman M, Lawinger P, Botas J, Bellen H. The Drosophila 18 wheeler is required for morphogenesis and has striking similarities to Toll. Development. 1994 Apr; 120(4):885-99. PMID: 7600965.
      Citations: 32     Fields:    Translation:AnimalsCells
    77. Capovilla M, Brandt M, Botas J. Direct regulation of decapentaplegic by Ultrabithorax and its role in Drosophila midgut morphogenesis. Cell. 1994 Feb 11; 76(3):461-75. PMID: 7906203.
      Citations: 51     Fields:    Translation:AnimalsCells
    78. Botas J. Control of morphogenesis and differentiation by HOM/Hox genes. Curr Opin Cell Biol. 1993 Dec; 5(6):1015-22. PMID: 7907490.
      Citations: 40     Fields:    Translation:HumansAnimals
    79. Irvine KD, Botas J, Jha S, Mann RS, Hogness DS. Negative autoregulation by Ultrabithorax controls the level and pattern of its expression. Development. 1993 Jan; 117(1):387-99. PMID: 7900988.
      Citations: 18     Fields:    Translation:Animals
    80. Vachon G, Cohen B, Pfeifle C, McGuffin ME, Botas J, Cohen SM. Homeotic genes of the Bithorax complex repress limb development in the abdomen of the Drosophila embryo through the target gene Distal-less. Cell. 1992 Oct 30; 71(3):437-50. PMID: 1358457.
      Citations: 85     Fields:    Translation:AnimalsCells
    81. Capdevila MP, Botas J, Garc?a-Bellido A. Genetic interactions between the Polycomb locus and the Antennapedia and Bithorax complexes of Drosophila. Rouxs Arch Dev Biol. 1986 Sep; 195(7):417-432. PMID: 28305404.
      Citations: 10     
    82. Morata G, Botas J, Kerridge S, Struhl G. Homeotic transformations of the abdominal segments of Drosophila caused by breaking or deleting a central portion of the bithorax complex. J Embryol Exp Morphol. 1983 Dec; 78:319-41. PMID: 6420494.
      Citations: 12     Fields:    Translation:Animals
    83. Botas J, Moscoso del Prado J, Garc?a-Bellido A. Gene-dose titration analysis in the search of trans-regulatory genes in Drosophila. EMBO J. 1982; 1(3):307-10. PMID: 7188185; PMCID: PMC553040.
      Citations: 47     Fields:    Translation:Animals
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