Author details

Full Name

Fen-Biao Gao

Affiliation

ORCID

Career Start Year

1993

Papers

129

H Index

Expertise

CM4AI Collaborator

PMID	Paper Title	Journal Title	Published Year
36849436	Non-muscle MYH10/myosin IIB recruits ESCRT-III to participate in autophagosome closure to maintain neuronal homeostasis.		2023
37675986	Translation of dipeptide repeat proteins in ALS/FTD through unique and redundant AUG initiation codons.		2023
37849894	Loss of TDP-43 function contributes to genomic instability in amyotrophic lateral sclerosis.	Front Neurosci	2023
37645251	Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43 mutation display a mild reactive state and release polyP toxic to motoneurons.		2023
37346371	Moderate intrinsic phenotypic alterations in ALS/FTD iPSC-microglia despite the presence of C9orf72 pathological features.		2023
37085629	The exocyst complex in neurological disorders.	Hum Genet	2023
36638803	Dorsomedial prefrontal hypoexcitability underlies lost empathy in frontotemporal dementia.		2023
36754049	PIKFYVE inhibition mitigates disease in models of diverse forms of ALS.		2023
36931278	Downregulation of Hsp90 and the antimicrobial peptide Mtk suppresses poly(GR)-induced neurotoxicity in C9ORF72-ALS/FTD.	Neuron	2023
35209956	RNA targets of TDP-43: Which one is more important in neurodegeneration?	Translational Neurodegeneration	2022
35589711	Poly(GR) and poly(GA) in cerebrospinal fluid as potential biomarkers for C9ORF72-ALS/FTD.	Nat Commun	2022
35589706	Ribosome inhibition by C9ORF72-ALS/FTD-associated poly-PR and poly-GR proteins revealed by cryo-EM.	Nat Commun	2022
36271076	CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro.	Nat Commun	2022
36414997	The enhanced association between mutant CHMP2B and spastin is a novel pathological link between frontotemporal dementia and hereditary spastic paraplegias.	Acta neuropathologica communications	2022
35276083	Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons.	Neuron	2022
34848561	Ribosome profiling reveals novel regulation of GGGGCC repeat-containing RNA translation.	RNA	2022
34654821	A C. elegans model of C9orf72-associated ALS/FTD uncovers a conserved role for eIF2D in RAN translation.	Nature Communications	2021
34081168	TBK1 haploinsufficiency in ALS and FTD compromises membrane trafficking.	Acta Neuropathologica	2021
33482083	p53 is a central regulator driving neurodegeneration caused by C9orf72 poly(PR).	Cell	2021
34157654	Translation of the poly(GR) frame in C9ORF72-ALS/FTD is regulated by cis-elements involved in alternative splicing.	Neurobiology of Aging	2021
32848189	Ik2/TBK1 and Hook/Dynein, an adaptor complex for early endosome transport, are genetic modifiers of FTD-associated mutant CHMP2B toxicity in Drosophila.	Scientific Reports	2020
32266467	CRISPR deletion of the C9ORF72 promoter in ALS/FTD patient motor neurons abolishes production of dipeptide repeat proteins and rescues neurodegeneration.	Acta Neuropathologica	2020
32178712	Premature termination codon readthrough upregulates progranulin expression and improves lysosomal function in preclinical models of GRN deficiency.	Mol Neurodegener	2020
32755582	Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD.	Cell Reports	2020
31019093	Partial inhibition of the overactivated Ku80-dependent DNA repair pathway rescues neurodegeneration in <i>C9ORF72</i>-ALS/FTD.	Proc Natl Acad Sci U S A	2019
31587919	CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation.	Neuron	2019
31784536	Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.	Nature Communications	2019
31624870	Production of poly(GA) in C9ORF72 patient motor neurons derived from induced pluripotent stem cells.	Acta Neuropathologica	2019
31086314	C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.	Nat Neurosci	2019
29729808	Insights into C9ORF72-Related ALS/FTD from Drosophila and iPSC Models.	Trends in Neurosciences	2018
29432529	The pro-apoptotic JNK scaffold POSH/SH3RF1 mediates CHMP2BIntron5-associated toxicity in animal models of frontotemporal dementia.	Human Molecular Genetics	2018
29373831	Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules.	Cell	2018
29501653	The epidemiology and genetics of Amyotrophic lateral sclerosis in China.	Brain Res	2018
28103472	Microsatellite Expansion Diseases: Repeat Toxicity Found in Translation.	Neuron	2017
28916614	Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.	EMBO Journal	2017
28379367	A Drosophila model of ALS reveals a partial loss of function of causative human PFN1 mutants.	Human Molecular Genetics	2017
28614712	Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.	Cell Rep	2017
28743268	The lysosomal protein cathepsin L is a progranulin protease.	Mol Neurodegener	2017
29149615	Rethinking Unconventional Translation in Neurodegeneration.	Cell	2017
27712723	Autophagy in neurodegenerative diseases.	Brain Research	2016
27154207	Lost & found: C9ORF72 and the autophagy pathway in ALS/FTD.	EMBO Journal	2016
27143419	Suberoylanilide hydroxamic acid increases progranulin production in iPSC-derived cortical neurons of frontotemporal dementia patients.	Neurobiol Aging	2016
26799652	Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).	Autophagy	2016
26869642	ALS-linked protein disulfide isomerase variants cause motor dysfunction.	EMBO Journal	2016
27369896	A Novel Protocol for Directed Differentiation of C9orf72-Associated Human Induced Pluripotent Stem Cells Into Contractile Skeletal Myotubes.	Stem Cells Transl Med	2016
27516603	Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts.	Science	2016
27594586	MMP-9 and MMP-2 Contribute to Neuronal Cell Death in iPSC Models of Frontotemporal Dementia with MAPT Mutations.	Stem Cell Reports	2016
27594585	Human iPSC-Derived Neuronal Model of Tau-A152T Frontotemporal Dementia Reveals Tau-Mediated Mechanisms of Neuronal Vulnerability.	Stem Cell Reports	2016
27720481	Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.	Neuron	2016
26031661	FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions.	Acta Neuropathologica	2015