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 Research Focus: Understanding ALS and Frontotemporal Dementia 

Dr. Fen-Biao GaoFen-Biao Gao Lab Website

Frontotemporal dementia (FTD) is an age-dependent neurodegenerative condition associated with focal atrophy of the frontal and/or temporal lobes and recognized now as the most common form of dementia before the age of 60. Unfortunately, the molecular pathogenesis of FTD remains largely unknown and effective treatments are not available. Recent exciting advances indicate that FTD is often associated with amyotrophic lateral sclerosis (ALS) and several genes are involved in thepathogenesis of both ALS and FTD, including CHMP2B, TDP-43, FUS, TBK1, and C9ORF72. How these mutant proteins cause or contribute to neuronal dysfunction and neurodegeneration in ALS and FTD remains poorly defined.

A few years ago, we cloned anovel Drosophila gene called shrub, which encodes a key component of ESCRT-III and regulates dendritic morphogenesis. In cultured cortical neurons, we found that dysfunctional ESCRT-III, lacking essential components (such as mSnf7-2, one of the mouse homologs of Shrub) or containing ectopically expressed FTD3-associated mutant CHMP2B, causes dendritic retraction,autophagosome accumulation and eventual neurodegeneration. Through an unbiased genetic screen in a Drosophila modelof FTD3, we identified several genetic modifiers of CHMP2B toxicity in vivo that are currently being characterized. We have also been using Drosophila models to study other ALS/FTD genes, in paticular, C9ORF72.

We have also established patient-specific induced pluripotent stem cells (iPSC) models of FTD and ALS with mutations in progranulin, TDP-43, C9ORF72 and other genes. Over the next a few years, we will use a combination of molecular, cellular, and genetic approaches in both Drosophila and iPSCs models to further dissect the pathogenic mechanisms involving these ALS/FTD disease genes. Our ultimate goal is to identify common underlying pathogenic pathways as potential targets for therapeutic interventions in both ALS and FTD.

One or more keywords matched the following items that are connected to Gao, Fen-Biao
Item TypeName
Academic Article Control of dendritic field formation in Drosophila: the roles of flamingo and competition between homologous neurons.
Academic Article Understanding fragile X syndrome: insights from retarded flies.
Academic Article Actin filament-stabilizing protein tropomyosin regulates the size of dendritic fields.
Academic Article The fragile X-related gene affects the crawling behavior of Drosophila larvae by regulating the mRNA level of the DEG/ENaC protein pickpocket1.
Academic Article Genes regulating dendritic outgrowth, branching, and routing in Drosophila.
Academic Article Abelson, enabled, and p120 catenin exert distinct effects on dendritic morphogenesis in Drosophila.
Academic Article MicroRNA-9a ensures the precise specification of sensory organ precursors in Drosophila.
Academic Article The steady-state level of the nervous-system-specific microRNA-124a is regulated by dFMR1 in Drosophila.
Academic Article Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching.
Academic Article Syntaxin 13, a genetic modifier of mutant CHMP2B in frontotemporal dementia, is required for autophagosome maturation.
Academic Article Genetic control of dendritic morphogenesis in Drosophila.
Academic Article Control of dendritic development by the Drosophila fragile X-related gene involves the small GTPase Rac1.
Academic Article BTB/POZ-zinc finger protein abrupt suppresses dendritic branching in a neuronal subtype-specific and dosage-dependent manner.
Academic Article Transcriptional control of dendritic patterning in Drosophila neurons.
Academic Article The Drosophila homologue of the Angelman syndrome ubiquitin ligase regulates the formation of terminal dendritic branches.
Academic Article Genetic screen identifies serpin5 as a regulator of the toll pathway and CHMP2B toxicity associated with frontotemporal dementia.
Concept Drosophila melanogaster
Concept Drosophila
Concept Drosophila Proteins
Academic Article Expression of mutant CHMP2B, an ESCRT-III component involved in frontotemporal dementia, causes eye deformities due to Notch misregulation in Drosophila.
Academic Article The FTD/ALS-associated RNA-binding protein TDP-43 regulates the robustness of neuronal specification through microRNA-9a in Drosophila
Academic Article Sequoia, a tramtrack-related zinc finger protein, functions as a pan-neural regulator for dendrite and axon morphogenesis in Drosophila
Academic Article The coiled-coil protein shrub controls neuronal morphogenesis in Drosophila
Academic Article Neurophysiological defects and neuronal gene deregulation in Drosophila mir-124 mutants
Academic Article Rab8, POSH, and TAK1 regulate synaptic growth in a Drosophila model of frontotemporal dementia.
Academic Article Downregulation of the Host Gene jigr1 by miR-92 Is Essential for Neuroblast Self-Renewal in Drosophila.
Academic Article FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions.
Academic Article Differential toxicity of nuclear RNA foci versus dipeptide repeat proteins in a Drosophila model of C9ORF72 FTD/ALS
Academic Article GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function.
Academic Article Genetic manipulation of single neurons in vivo reveals specific roles of flamingo in neuronal morphogenesis.
Academic Article Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts.
Academic Article A Drosophila model of ALS reveals a partial loss of function of causative human PFN1 mutants.
Academic Article Insights into C9ORF72-Related ALS/FTD from Drosophila and iPSC Models.
Academic Article The pro-apoptotic JNK scaffold POSH/SH3RF1 mediates CHMP2BIntron5-associated toxicity in animal models of frontotemporal dementia.
Academic Article Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules.
Academic Article Partial inhibition of the overactivated Ku80-dependent DNA repair pathway rescues neurodegeneration in C9ORF72-ALS/FTD.
Academic Article Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.
Academic Article Ik2/TBK1 and Hook/Dynein, an adaptor complex for early endosome transport, are genetic modifiers of FTD-associated mutant CHMP2B toxicity in Drosophila.
Academic Article p53 is a central regulator driving neurodegeneration caused by C9orf72 poly(PR).
Academic Article CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation.
Academic Article Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD.
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