Published in J Neurogenet on February 10, 2014
Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS. EMBO J (2015) 0.88
The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease. Nat Rev Neurosci (2016) 0.84
Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis. Neurotherapeutics (2015) 0.83
Role of FET proteins in neurodegenerative disorders. RNA Biol (2016) 0.79
Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets? Neurodegener Dis Manag (2014) 0.78
Exploring the genetic underpinnings of brain and behavioral disorders. J Neurogenet (2014) 0.77
The role of CHMP2B(Intron5) in autophagy and frontotemporal dementia. Brain Res (2016) 0.75
The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine. Stem Cells Int (2016) 0.75
Noncoding RNAs in neurodegeneration. Nat Rev Neurosci (2017) 0.75
The C. elegans heterochronic gene lin-4 encodes small RNAs with antisense complementarity to lin-14. Cell (1993) 64.62
Posttranscriptional regulation of the heterochronic gene lin-14 by lin-4 mediates temporal pattern formation in C. elegans. Cell (1993) 33.79
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
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
The Microprocessor complex mediates the genesis of microRNAs. Nature (2004) 17.70
The widespread regulation of microRNA biogenesis, function and decay. Nat Rev Genet (2010) 15.87
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science (2008) 14.09
Alzheimer's disease is a synaptic failure. Science (2002) 13.82
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
Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature (2006) 11.59
Non-coding RNAs in human disease. Nat Rev Genet (2011) 11.40
microRNAs: tiny regulators with great potential. Cell (2001) 11.23
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun (2006) 10.69
Gene silencing by microRNAs: contributions of translational repression and mRNA decay. Nat Rev Genet (2011) 9.79
Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron (2010) 7.87
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet (2004) 7.84
Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription. Nature (2008) 7.18
Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat Rev Neurosci (2006) 6.53
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (2013) 6.34
Pervasive roles of microRNAs in cardiovascular biology. Nature (2011) 6.08
Multivesicular bodies associate with components of miRNA effector complexes and modulate miRNA activity. Nat Cell Biol (2009) 6.03
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91
MicroRNA control of signal transduction. Nat Rev Mol Cell Biol (2010) 5.74
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3. Brain (2006) 5.36
A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia. Neurology (2006) 5.29
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature (2010) 5.26
Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping. EMBO J (2001) 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
MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice. Science (2009) 5.17
Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration. Hum Mol Genet (2006) 4.80
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature (2011) 4.56
Cerebellar neurodegeneration in the absence of microRNAs. J Exp Med (2007) 4.48
A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain (2009) 4.40
Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. J Cell Biol (2007) 4.30
Membrane budding and scission by the ESCRT machinery: it's all in the neck. Nat Rev Mol Cell Biol (2010) 4.29
Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet (2005) 4.21
The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology (2002) 3.91
p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS. Acta Neuropathol (2011) 3.73
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Non-coding RNAs as regulators of embryogenesis. Nat Rev Genet (2011) 3.36
Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol (2011) 3.34
Conditional loss of Dicer disrupts cellular and tissue morphogenesis in the cortex and hippocampus. J Neurosci (2008) 3.33
ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration. Curr Biol (2007) 3.27
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron (2013) 3.25
Silencing by small RNAs is linked to endosomal trafficking. Nat Cell Biol (2009) 3.25
MicroRNAs in human cancer. Adv Exp Med Biol (2013) 3.19
The changing scene of amyotrophic lateral sclerosis. Nat Rev Neurosci (2013) 3.16
VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD. Autophagy (2010) 2.99
Frontotemporal dementia. Lancet Neurol (2005) 2.96
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
The microRNA miR-34 modulates ageing and neurodegeneration in Drosophila. Nature (2012) 2.94
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med (2013) 2.89
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Proc Natl Acad Sci U S A (2010) 2.65
The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology. Curr Biol (2005) 2.53
MicroRNAs and gene regulatory networks: managing the impact of noise in biological systems. Genes Dev (2010) 2.51
Dicer inactivation leads to progressive functional and structural degeneration of the mouse retina. J Neurosci (2008) 2.50
Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia. Hum Mol Genet (2008) 2.48
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48
Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry (2010) 2.44
TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling. J Cell Sci (1997) 2.35
Dicer loss in striatal neurons produces behavioral and neuroanatomical phenotypes in the absence of neurodegeneration. Proc Natl Acad Sci U S A (2008) 2.32
Pathogenic mechanisms of myotonic dystrophy. Biochem Soc Trans (2009) 2.32
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia. Proc Natl Acad Sci U S A (2013) 2.31
Neuronal activity rapidly induces transcription of the CREB-regulated microRNA-132, in vivo. Hippocampus (2010) 2.30
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol (2013) 2.26
Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy. Hum Mol Genet (2010) 2.25
Oncoprotein TLS interacts with serine-arginine proteins involved in RNA splicing. J Biol Chem (1998) 2.21
Genetic contribution of FUS to frontotemporal lobar degeneration. Neurology (2010) 2.14
VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder. Biochim Biophys Acta (2008) 2.06
ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B). Neurology (2006) 2.00
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS. Acta Neuropathol (2013) 1.95
Selective autophagy degrades DICER and AGO2 and regulates miRNA activity. Nat Cell Biol (2012) 1.91
TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes. Proc Natl Acad Sci U S A (2012) 1.91
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function. Cell (2013) 1.88
miRNA malfunction causes spinal motor neuron disease. Proc Natl Acad Sci U S A (2010) 1.83
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins. Acta Neuropathol (2013) 1.82
Mutation within TARDBP leads to frontotemporal dementia without motor neuron disease. Hum Mutat (2009) 1.77
Context-dependent functions of specific microRNAs in neuronal development. Neural Dev (2010) 1.74
Variations in the progranulin gene affect global gene expression in frontotemporal lobar degeneration. Hum Mol Genet (2008) 1.68
Nuclear factor TDP-43 can affect selected microRNA levels. FEBS J (2010) 1.66
MicroRNAs shape the neuronal landscape. Neuron (2012) 1.64
TMEM106B, the risk gene for frontotemporal dementia, is regulated by the microRNA-132/212 cluster and affects progranulin pathways. J Neurosci (2012) 1.60
Molecular mechanisms of the membrane sculpting ESCRT pathway. Cold Spring Harb Perspect Biol (2013) 1.58
Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome. Cell Rep (2013) 1.56
miR-107 regulates granulin/progranulin with implications for traumatic brain injury and neurodegenerative disease. Am J Pathol (2010) 1.54
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci. Acta Neuropathol (2013) 1.51
Differential gene expression in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2011) 1.48
Inhibition of autophagy induction delays neuronal cell loss caused by dysfunctional ESCRT-III in frontotemporal dementia. J Neurosci (2009) 1.45
Involvement of the pro-oncoprotein TLS (translocated in liposarcoma) in nuclear factor-kappa B p65-mediated transcription as a coactivator. J Biol Chem (2001) 1.45
The Ataxin-2 protein is required for microRNA function and synapse-specific long-term olfactory habituation. Proc Natl Acad Sci U S A (2011) 1.43
miR-132, an experience-dependent microRNA, is essential for visual cortex plasticity. Nat Neurosci (2011) 1.40
MicroRNAs and neurodegeneration: role and impact. Trends Cell Biol (2012) 1.38
FUS stimulates microRNA biogenesis by facilitating co-transcriptional Drosha recruitment. EMBO J (2012) 1.35
Functional genomic analyses identify pathways dysregulated by progranulin deficiency, implicating Wnt signaling. Neuron (2011) 1.34
The ubiquitin-associated domain of hPLIC-2 interacts with the proteasome. Mol Biol Cell (2003) 1.32
Experience-dependent expression of miR-132 regulates ocular dominance plasticity. Nat Neurosci (2011) 1.32
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy (2007) 20.92
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
MicroRNA-9a ensures the precise specification of sensory organ precursors in Drosophila. Genes Dev (2006) 6.30
ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration. Curr Biol (2007) 3.27
MicroRNA-9 coordinates proliferation and migration of human embryonic stem cell-derived neural progenitors. Cell Stem Cell (2010) 3.18
Midbody accumulation through evasion of autophagy contributes to cellular reprogramming and tumorigenicity. Nat Cell Biol (2011) 2.65
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol (2013) 2.26
Control of dendritic development by the Drosophila fragile X-related gene involves the small GTPase Rac1. Development (2003) 1.91
Suberoylanilide hydroxamic acid (vorinostat) up-regulates progranulin transcription: rational therapeutic approach to frontotemporal dementia. J Biol Chem (2011) 1.88
Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching. Mol Brain (2009) 1.65
The fragile X-related gene affects the crawling behavior of Drosophila larvae by regulating the mRNA level of the DEG/ENaC protein pickpocket1. Curr Biol (2004) 1.57
The coiled-coil protein shrub controls neuronal morphogenesis in Drosophila. Curr Biol (2006) 1.46
Inhibition of autophagy induction delays neuronal cell loss caused by dysfunctional ESCRT-III in frontotemporal dementia. J Neurosci (2009) 1.45
BTB/POZ-zinc finger protein abrupt suppresses dendritic branching in a neuronal subtype-specific and dosage-dependent manner. Neuron (2004) 1.41
The steady-state level of the nervous-system-specific microRNA-124a is regulated by dFMR1 in Drosophila. J Neurosci (2008) 1.38
MicroRNA-9: functional evolution of a conserved small regulatory RNA. RNA Biol (2011) 1.32
Genetic manipulation of single neurons in vivo reveals specific roles of flamingo in neuronal morphogenesis. Dev Biol (2002) 1.30
Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects. Cell Rep (2012) 1.16
The advantages of frontotemporal degeneration drug development (part 2 of frontotemporal degeneration: the next therapeutic frontier). Alzheimers Dement (2012) 1.14
Posttranscriptional regulation of p18 and p27 Cdk inhibitor proteins and the timing of oligodendrocyte differentiation. Dev Biol (2002) 1.12
MicroRNA-29b regulates the expression level of human progranulin, a secreted glycoprotein implicated in frontotemporal dementia. PLoS One (2010) 1.11
Roles of ESCRT in autophagy-associated neurodegeneration. Autophagy (2007) 1.09
Cause or Effect: Misregulation of microRNA Pathways in Neurodegeneration. Front Neurosci (2012) 1.09
The Drosophila homologue of the Angelman syndrome ubiquitin ligase regulates the formation of terminal dendritic branches. Hum Mol Genet (2008) 1.08
Dissociation of frontotemporal dementia-related deficits and neuroinflammation in progranulin haploinsufficient mice. J Neurosci (2013) 1.06
Genetic screen identifies serpin5 as a regulator of the toll pathway and CHMP2B toxicity associated with frontotemporal dementia. Proc Natl Acad Sci U S A (2009) 1.04
Abelson, enabled, and p120 catenin exert distinct effects on dendritic morphogenesis in Drosophila. Dev Dyn (2005) 1.00
Patterns and functions of STAT activation during Drosophila embryogenesis. Mech Dev (2003) 0.99
FXR1P but not FMRP regulates the levels of mammalian brain-specific microRNA-9 and microRNA-124. J Neurosci (2011) 0.97
Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations. PLoS One (2013) 0.97
Frontotemporal degeneration, the next therapeutic frontier: molecules and animal models for frontotemporal degeneration drug development. Alzheimers Dement (2012) 0.96
Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Hum Mol Genet (2013) 0.93
Actin filament-stabilizing protein tropomyosin regulates the size of dendritic fields. J Neurosci (2003) 0.92
Autophagy defects contribute to neurodegeneration induced by dysfunctional ESCRT-III. Autophagy (2009) 0.90
Progranulin, a glycoprotein deficient in frontotemporal dementia, is a novel substrate of several protein disulfide isomerase family proteins. PLoS One (2011) 0.88
Neurophysiological defects and neuronal gene deregulation in Drosophila mir-124 mutants. PLoS Genet (2012) 0.87
Fluorescein analogues inhibit SecA ATPase: the first sub-micromolar inhibitor of bacterial protein translocation. ChemMedChem (2012) 0.86
The FTD/ALS-associated RNA-binding protein TDP-43 regulates the robustness of neuronal specification through microRNA-9a in Drosophila. Hum Mol Genet (2012) 0.86
ESCRT, autophagy, and frontotemporal dementia. BMB Rep (2008) 0.85
ESCRT-III subunits Snf7-1 and Snf7-2 differentially regulate transmembrane cargos in hESC-derived human neurons. Mol Brain (2011) 0.85
microRNA-9 multitasking near organizing centers. Nat Neurosci (2008) 0.84
Regulation of Abeta pathology by beclin 1: a protective role for autophagy? J Clin Invest (2008) 0.84
Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS. Neurobiol Dis (2012) 0.81
RNA-binding proteins in neurological disease. Brain Res (2012) 0.78
Neuronal Functions of ESCRTs. Exp Neurobiol (2012) 0.77
A new role for microRNA-9 in human neural progenitor cells. Cell Cycle (2010) 0.77
Expression of mutant CHMP2B, an ESCRT-III component involved in frontotemporal dementia, causes eye deformities due to Notch misregulation in Drosophila. FASEB J (2013) 0.76
Transcriptional control of dendritic patterning in Drosophila neurons. Genome Biol (2006) 0.75
Protocols for investigating microRNA functions in human neural progenitor cells. Methods Mol Biol (2012) 0.75