Drosophila as an In Vivo Model for Human Neurodegenerative Disease.

PubWeight™: 0.83‹?›

🔗 View Article (PMID 26447127)

Published in Genetics on October 01, 2015

Authors

Leeanne McGurk1, Amit Berson1, Nancy M Bonini2

Author Affiliations

1: Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104.
2: Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104 nbonini@sas.upenn.edu.

Articles cited by this

(truncated to the top 100)

Targeted gene expression as a means of altering cell fates and generating dominant phenotypes. Development (1993) 58.26

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96

Comparative genomics of the eukaryotes. Science (2000) 26.62

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature (1993) 21.65

alpha-Synuclein locus triplication causes Parkinson's disease. Science (2003) 20.20

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron (2011) 20.15

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron (2011) 18.73

A genome-wide transgenic RNAi library for conditional gene inactivation in Drosophila. Nature (2007) 18.38

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet (2004) 14.31

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (2009) 13.45

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (2009) 12.99

Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease. Nat Genet (1998) 12.92

Drosophila pink1 is required for mitochondrial function and interacts genetically with parkin. Nature (2006) 11.91

Mitochondrial dysfunction in Drosophila PINK1 mutants is complemented by parkin. Nature (2006) 11.81

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science (1997) 11.53

The new mutation, E46K, of alpha-synuclein causes Parkinson and Lewy body dementia. Ann Neurol (2004) 9.62

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell (1997) 9.57

Trinucleotide repeat disorders. Annu Rev Neurosci (2007) 9.45

alpha-Synuclein in filamentous inclusions of Lewy bodies from Parkinson's disease and dementia with lewy bodies. Proc Natl Acad Sci U S A (1998) 8.41

A Drosophila model of Parkinson's disease. Nature (2000) 7.82

RNA granules. J Cell Biol (2006) 7.79

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature (2007) 7.74

Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants. Proc Natl Acad Sci U S A (2003) 7.57

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat Genet (1994) 7.07

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature (2001) 6.76

Parkinson's disease. Lancet (2009) 6.60

Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy. EMBO J (2000) 6.56

The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (2013) 6.34

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell (1998) 6.24

Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease. Science (2001) 6.15

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91

Mitochondrial pathology and muscle and dopaminergic neuron degeneration caused by inactivation of Drosophila Pink1 is rescued by Parkin. Proc Natl Acad Sci U S A (2006) 5.78

A modular misexpression screen in Drosophila detecting tissue-specific phenotypes. Proc Natl Acad Sci U S A (1996) 5.42

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature (2010) 5.26

Biological and chemical approaches to diseases of proteostasis deficiency. Annu Rev Biochem (2009) 5.23

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol (2012) 5.18

A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study. Lancet Neurol (2011) 5.01

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat Genet (1999) 4.93

A conditional tissue-specific transgene expression system using inducible GAL4. Proc Natl Acad Sci U S A (2001) 4.93

Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71

A GAL4-driver line resource for Drosophila neurobiology. Cell Rep (2012) 4.67

Oral treatment targeting the unfolded protein response prevents neurodegeneration and clinical disease in prion-infected mice. Sci Transl Med (2013) 4.62

Tiling of the Drosophila epidermis by multidendritic sensory neurons. Development (2002) 4.59

Therapeutic application of histone deacetylase inhibitors for central nervous system disorders. Nat Rev Drug Discov (2008) 4.43

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat Genet (1998) 4.27

Tauopathy in Drosophila: neurodegeneration without neurofibrillary tangles. Science (2001) 4.25

RNA-mediated neuromuscular disorders. Annu Rev Neurosci (2006) 4.24

A genome-scale shRNA resource for transgenic RNAi in Drosophila. Nat Methods (2011) 4.24

Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat Genet (1996) 4.16

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science (2001) 4.13

Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell (1998) 4.07

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature (2013) 4.03

Identification of genes that modify ataxin-1-induced neurodegeneration. Nature (2000) 4.02

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A (2000) 4.01

The conserved microRNA miR-8 tunes atrophin levels to prevent neurodegeneration in Drosophila. Cell (2007) 4.00

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurol (2010) 3.92

The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology (2002) 3.91

The art and design of genetic screens: Drosophila melanogaster. Nat Rev Genet (2002) 3.86

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron (1997) 3.73

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron (1998) 3.72

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63

Non-ATG-initiated translation directed by microsatellite expansions. Proc Natl Acad Sci U S A (2010) 3.58

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. EMBO J (2010) 3.58

Sustained translational repression by eIF2α-P mediates prion neurodegeneration. Nature (2012) 3.54

State of play in amyotrophic lateral sclerosis genetics. Nat Neurosci (2013) 3.54

FlyBase 102--advanced approaches to interrogating FlyBase. Nucleic Acids Res (2013) 3.46

ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. Cell (2006) 3.39

From the gene to behavior. JAMA (1971) 3.30

Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2. Hum Mol Genet (2001) 3.27

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nat Genet (1996) 3.26

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron (2013) 3.25

Genetic suppression of polyglutamine toxicity in Drosophila. Science (2000) 3.23

Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons. Hum Mol Genet (2004) 3.21

17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med (2005) 3.19

Drosophila, the golden bug, emerges as a tool for human genetics. Nat Rev Genet (2005) 3.17

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nat Genet (1996) 3.07

Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron (2002) 2.98

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration. Proc Natl Acad Sci U S A (2013) 2.95

Design principles of insect and vertebrate visual systems. Neuron (2010) 2.94

The microRNA miR-34 modulates ageing and neurodegeneration in Drosophila. Nature (2012) 2.94

RNA-mediated neurodegeneration caused by the fragile X premutation rCGG repeats in Drosophila. Neuron (2003) 2.81

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins. Science (2014) 2.78

RNA toxicity is a component of ataxin-3 degeneration in Drosophila. Nature (2008) 2.74

A systematic analysis of human disease-associated gene sequences in Drosophila melanogaster. Genome Res (2001) 2.73

Clinical and pathological continuum of multisystem TDP-43 proteinopathies. Arch Neurol (2009) 2.68

A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A (2011) 2.68

Drosophila as a model for human neurodegenerative disease. Annu Rev Genet (2005) 2.63

TDP-43 is recruited to stress granules in conditions of oxidative insult. J Neurochem (2009) 2.57

Dissecting the pathological effects of human Abeta40 and Abeta42 in Drosophila: a potential model for Alzheimer's disease. Proc Natl Acad Sci U S A (2004) 2.56

MicroRNA pathways modulate polyglutamine-induced neurodegeneration. Mol Cell (2006) 2.55

Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior. FEBS Lett (2009) 2.48

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat Rev Neurosci (2000) 2.44

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice. Neuron (2003) 2.39

A Drosophila model for TDP-43 proteinopathy. Proc Natl Acad Sci U S A (2010) 2.36

Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. J Proteome Res (2010) 2.35

Suppression of eIF2α kinases alleviates Alzheimer's disease-related plasticity and memory deficits. Nat Neurosci (2013) 2.35

RNA structure of trinucleotide repeats associated with human neurological diseases. Nucleic Acids Res (2003) 2.34

The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions. Brain (2012) 2.33

The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs. Bioinformatics (2013) 2.31

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia. Proc Natl Acad Sci U S A (2013) 2.31