title: Studying polyglutamine diseases in Drosophila.
abstract: Polyglutamine (polyQ) diseases are a family of dominantly transmitted neurodegenerative disorders caused by an abnormal expansion of CAG trinucleotide repeats in the protein-coding regions of the respective disease-causing genes. Despite their simple genetic basis, the etiology of these diseases is far from clear. Over the past two decades, Drosophila has proven to be successful in modeling this family of neurodegenerative disorders, including the faithful recapitulation of pathological features such as polyQ length-dependent formation of protein aggregates and progressive neuronal degeneration. Additionally, it has been valuable in probing the pathogenic mechanisms, in identifying and evaluating disease modifiers, and in helping elucidate the normal functions of disease-causing genes. Knowledge learned from this simple invertebrate organism has had a large impact on our understanding of these devastating brain diseases.
authors.affiliation: The Brown Foundation Institute of Molecular Medicine, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Medical School at Houston, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Health Science Center at Houston (UTHealth), 1825 Pressler Street, Houston, TX 77030, United States.
authors.forename: Zhen
authors.initials: Z
authors.lastname: Xu
authors.affiliation: The Brown Foundation Institute of Molecular Medicine, 1825 Pressler Street, Houston, TX 77030, United States; Programs in Human and Molecular Genetics and Neuroscience, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Graduate School of Biomedical Sciences, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Medical School at Houston, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Health Science Center at Houston (UTHealth), 1825 Pressler Street, Houston, TX 77030, United States.
authors.forename: Antonio Joel
authors.initials: AJ
authors.lastname: Tito
authors.affiliation: The Brown Foundation Institute of Molecular Medicine, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Medical School at Houston, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Health Science Center at Houston (UTHealth), 1825 Pressler Street, Houston, TX 77030, United States.
authors.forename: Yan-Ning
authors.initials: YN
authors.lastname: Rui
authors.affiliation: The Brown Foundation Institute of Molecular Medicine, 1825 Pressler Street, Houston, TX 77030, United States; Department of Neurobiology and Anatomy, 1825 Pressler Street, Houston, TX 77030, United States; Programs in Human and Molecular Genetics and Neuroscience, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Graduate School of Biomedical Sciences, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Medical School at Houston, 1825 Pressler Street, Houston, TX 77030, United States; The University of Texas Health Science Center at Houston (UTHealth), 1825 Pressler Street, Houston, TX 77030, United States. Electronic address: sheng.zhang@uth.tmc.edu.
authors.forename: Sheng
authors.initials: S
authors.lastname: Zhang
mesh_terms: D000818:Animals; D004330:Drosophila; D029721:Drosophila Proteins / Q000235:genetics*; D006801:Humans; D009419:Nerve Tissue Proteins / Q000235:genetics; D019636:Neurodegenerative Diseases / Q000235:genetics* / Q000473:pathology; D010455:Peptides / Q000235:genetics*; D018911:Trinucleotide Repeats / Q000235:genetics
chemical_list: D029721:Drosophila Proteins; D009419:Nerve Tissue Proteins; D010455:Peptides; C097188:polyglutamine
keywords: Atrophin-1; DRPLA; Dentatorubral-pallidoluysian atrophy; Drosophila model; HD; HTT; Huntingtin; Huntington's disease; Machado–Joseph disease; PolyQ diseases; Polyglutamine diseases; SBMA; SCA1; Spinobulbar muscular atrophy; Spinocerebellar ataxia