Juan Botas

Summary

Affiliation: Texas Medical Center
Country: USA

Publications

  1. ncbi request reprint Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases
    Joana Branco
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Hum Mol Genet 17:376-90. 2008
  2. ncbi request reprint Drosophila researchers focus on human disease
    Juan Botas
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA
    Nat Genet 39:589-91. 2007
  3. pmc dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1
    Ismael Al-Ramahi
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 3:e234. 2007
  4. pmc RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS
    Oyinkan A Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Neuron 55:565-71. 2007
  5. pmc The Drosophila FMRP and LARK RNA-binding proteins function together to regulate eye development and circadian behavior
    Oyinkan Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    J Neurosci 28:10200-5. 2008
  6. pmc Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm
    Eliana Romero
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Neuron 57:27-40. 2008
  7. ncbi request reprint ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology
    Yung C Lam
    Department of Neuroscience, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 127:1335-47. 2006
  8. pmc RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1
    Jeehye Park
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    Nature 498:325-31. 2013
  9. pmc Genetic modifiers of MeCP2 function in Drosophila
    HOLLY N CUKIER
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 4:e1000179. 2008
  10. ncbi request reprint Argonaute-2-dependent rescue of a Drosophila model of FXTAS by FRAXE premutation repeat
    Oyinkan A Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 16:2326-32. 2007

Research Grants

  1. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2010
  2. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2003
  3. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2004
  4. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2004
  5. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2005
  6. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2004
  7. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2005
  8. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2009
  9. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2009
  10. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2003

Collaborators

  • S E Celniker
  • Huda Y Zoghbi
  • David L Nelson
  • Peng Jin
  • H T Orr
  • Robia G Pautler
  • Robert E Hughes
  • Pedro Fernandez-Funez
  • Cindy V Ly
  • Sarita Lagalwar
  • F Rob Jackson
  • Patrik Verstreken
  • E M C Skoulakis
  • Casey M Bergman
  • Mark Stapleton
  • R A Hoskins
  • Ismael Al-Ramahi
  • Maria de Haro
  • Alma M Perez
  • Jerrah K Holth
  • Joana Branco
  • Oyinkan A Sofola
  • Yung C Lam
  • Hung Kai Chen
  • Eliana Romero
  • Lubna Ukani
  • Jeehye Park
  • Paymaan Jafar-Nejad
  • Ronald Richman
  • Oyinkan Sofola
  • HOLLY N CUKIER
  • Guang Ho Cha
  • Linda S Kaltenbach
  • Minghang Zhang
  • Hugo J Bellen
  • Janghoo Lim
  • Beatrice de Gouyon
  • Hiroshi Tsuda
  • Lubov T Timchenko
  • Yoontae Lee
  • J Graham Reed
  • J Simon C Arthur
  • Nissa Mollema
  • Lisa Duvick
  • Hsiang Chih Lu
  • Taeko Inoue
  • Layal S Sayegh
  • Yan Gao
  • Karlie R Jones
  • Thomas F Westbrook
  • Qiumin Tan
  • Valerie C Bomben
  • Steven G Younkin
  • Hyojin Kang
  • Tatiana Gallego-Flores
  • Chad A Shaw
  • Javier R Diaz-Garcia
  • Jeffrey L Noebels
  • Linda Younkin
  • Xiuyun Liu
  • Yelena Kleyner
  • Ann L Collins
  • Fanny Ng
  • Joannella Morales
  • Vasudha Sundram
  • Zhaolan Zhou
  • Diego Rinc√≥n-Limas
  • Rakesh Chettier
  • Stefan M Pulst
  • Sudhir Sahasrabudhe
  • Rie Sorensen
  • Russell Bell
  • Justin Savage
  • Ranhui Duan
  • Cameron Torcassi
  • Anthony Hurlburt
  • John M Peltier
  • Amit Phansalkar
  • Yunlong Qin
  • Yuejun Zhen
  • Lisa M Ellerby
  • James Olson
  • Andrew Strand
  • Cornelia Kurschner
  • Huijie Liu
  • Robert R Becklin
  • Cindy Lou Chepanoske
  • Cam Patterson
  • Eric D Hyun
  • John D Fryer

Detail Information

Publications19

  1. ncbi request reprint Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases
    Joana Branco
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Hum Mol Genet 17:376-90. 2008
    ..Our results point to potential common therapeutic targets in novel pathways, and to genes and pathways responsible for differences between Ataxin-1 and Huntingtin-induced neurodegeneration...
  2. ncbi request reprint Drosophila researchers focus on human disease
    Juan Botas
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA
    Nat Genet 39:589-91. 2007
  3. pmc dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1
    Ismael Al-Ramahi
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 3:e234. 2007
    ..Altogether, these findings reveal a previously unknown functional link between neurodegenerative disorders with common clinical features but different etiology...
  4. pmc RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS
    Oyinkan A Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Neuron 55:565-71. 2007
    ..Furthermore, we show that hnRNP A2/B1 directly interacts with riboCGG repeats and that the CUGBP1 protein interacts with the riboCGG repeats via hnRNP A2/B1...
  5. pmc The Drosophila FMRP and LARK RNA-binding proteins function together to regulate eye development and circadian behavior
    Oyinkan Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    J Neurosci 28:10200-5. 2008
    ..Furthermore, we show genetic interactions between the corresponding genes indicating that dFMRP and LARK function together to regulate eye development and circadian behavior...
  6. pmc Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm
    Eliana Romero
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Neuron 57:27-40. 2008
    ..Thus, our data indicate that increased neurotransmission is at the root of neuronal degeneration caused by expanded full-length htt during early stages of pathogenesis...
  7. ncbi request reprint ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology
    Yung C Lam
    Department of Neuroscience, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 127:1335-47. 2006
    ..These data provide insight into the function of ATXN1 and suggest that SCA1 neuropathology depends on native, not novel, protein interactions...
  8. pmc RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1
    Jeehye Park
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    Nature 498:325-31. 2013
    ..Collectively, these data reveal new therapeutic entry points for SCA1 and provide a proof-of-principle for tackling other classes of intractable neurodegenerative diseases...
  9. pmc Genetic modifiers of MeCP2 function in Drosophila
    HOLLY N CUKIER
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 4:e1000179. 2008
    ..These findings demonstrate that anatomical and behavioral phenotypes caused by MeCP2 activity can be ameliorated by altering other factors that might be more amenable to manipulation than MeCP2 itself...
  10. ncbi request reprint Argonaute-2-dependent rescue of a Drosophila model of FXTAS by FRAXE premutation repeat
    Oyinkan A Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 16:2326-32. 2007
    ..These data support the concept that RNA toxicity is the mechanism of neuronal toxicity and suggests potential reversal of RNA-mediated phenotypes with complementary RNA molecules...
  11. ncbi request reprint CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation
    Ismael Al-Ramahi
    Department of Molecular and Human Genetics, Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 281:26714-24. 2006
    ..These data underscore the importance of the protein framework for modulating the effects of polyglutamine-induced neurodegeneration...
  12. pmc Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy
    Maria de Haro
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 9:e1003445. 2013
    ..Therefore, Smaug suppresses CUG-mediated muscle wasting at least in part via restoration of translational activity of CUGBP1...
  13. pmc Tau loss attenuates neuronal network hyperexcitability in mouse and Drosophila genetic models of epilepsy
    Jerrah K Holth
    Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Neurosci 33:1651-9. 2013
    ....
  14. ncbi request reprint MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1
    Maria de Haro
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 15:2138-45. 2006
    ..Perhaps more importantly, they also provide proof of the principle that CUG-induced muscle toxicity can be suppressed...
  15. ncbi request reprint The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins
    Hiroshi Tsuda
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    Cell 122:633-44. 2005
    ..Interestingly, loss of Gfi-1 mimics SCA1 phenotypes in Purkinje cells. These results indicate that the Atx-1/Gfi-1 interaction contributes to the selective Purkinje cell degeneration in SCA1...
  16. ncbi request reprint Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1
    Hung Kai Chen
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 113:457-68. 2003
    ..Our finding that phosphatidylinositol 3-kinase/Akt signaling and 14-3-3 cooperate to modulate the neurotoxicity of ataxin-1 provides insight into SCA1 pathogenesis and identifies potential targets for therapeutic intervention...
  17. ncbi request reprint Mouse and fly models of neurodegeneration
    Huda Y Zoghbi
    Depts of Pediatrics, Neurology, and Molecular and Human Genetics, and Howard Hughes Medical Institute, Baylor College of Medicine, 77030, Houston, TX, USA
    Trends Genet 18:463-71. 2002
    ..In each case, we will stress what has been learned about the role of protein clearance and the questions that remain about how misfolded proteins acquire their toxicity...
  18. pmc Huntingtin interacting proteins are genetic modifiers of neurodegeneration
    Linda S Kaltenbach
    Prolexys Pharmaceuticals, Salt Lake City, Utah, United States of America
    PLoS Genet 3:e82. 2007
    ..These studies demonstrate that high-throughput screening for protein interactions combined with genetic validation in a model organism is a powerful approach for identifying novel candidate modifiers of polyglutamine toxicity...
  19. pmc Assessing the impact of comparative genomic sequence data on the functional annotation of the Drosophila genome
    Casey M Bergman
    Berkeley Drosophila Genome Project, Lawrence Berkeley National Laboratory, One Cyclotron Road, Berkeley, CA 94720, USA
    Genome Biol 3:RESEARCH0086. 2002
    ..It is widely accepted that comparative sequence data can aid the functional annotation of genome sequences; however, the most informative species and features of genome evolution for comparison remain to be determined...

Research Grants22

  1. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2010
    ..These basic studies are a prerequisite to developing therapies for these neurodegenerative disorders for which there are no effective treatments. ..
  2. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2003
    ..Many of these genes are implicated in human developmental malformations and in an increasing number of human diseases. These include different types of cancer, stroke and dementia. ..
  3. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2004
    ..Many of these genes are implicated in human developmental malformations and in an increasing number of human diseases. These include different types of cancer, stroke and dementia. ..
  4. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2004
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  5. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2005
    ..Many of these genes are implicated in human developmental malformations and in an increasing number of human diseases. These include different types of cancer, stroke and dementia. ..
  6. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2004
    ..Many of these genes are implicated in human developmental malformations and in an increasing number of human diseases. These include different types of cancer, stroke and dementia. ..
  7. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2005
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  8. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2009
    ..These basic studies are a prerequisite to developing therapies for these neurodegenerative disorders for which there are no effective treatments. ..
  9. Probing Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2009
    ..These basic studies are a prerequisite to developing therapies for these neurodegenerative disorders for which there are no effective treatments. ..
  10. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2003
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  11. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2002
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  12. STRUCTURE/FUNCTION STUDIES ON DROSOPHILA APTEROUS
    Juan Botas; Fiscal Year: 1999
    ..no abstract available ..
  13. STRUCTURE/FUNCTION STUDIES ON DROSOPHILA APTEROUS
    Juan Botas; Fiscal Year: 1999
    ..no abstract available ..
  14. STRUCTURE/FUNCTION STUDIES ON DROSOPHILA APTEROUS
    Juan Botas; Fiscal Year: 2000
    ..no abstract available ..
  15. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2001
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  16. STRUCTURE/FUNCTION STUDIES ON DROSOPHILA APTEROUS
    Juan Botas; Fiscal Year: 2001
    ..no abstract available ..
  17. A genetic analysis of Drosophila limb patterning
    Juan Botas; Fiscal Year: 2002
    ..Many of these genes are implicated in human developmental malformations and in an increasing number of human diseases. These include different types of cancer, stroke and dementia. ..
  18. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2001
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..
  19. Neurodegeneration with Drosophila
    Juan Botas; Fiscal Year: 2002
    ..They will provide valuable targets for future pharmacological research aimed at developing drugs for therapy. ..