Published in Ann Neurol on July 01, 2006
Hereditary spastic paraplegias: membrane traffic and the motor pathway. Nat Rev Neurosci (2011) 2.30
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A (2008) 1.84
Action in the axon: generation and transport of signaling endosomes. Curr Opin Neurobiol (2008) 1.69
Rho guanine nucleotide exchange factors: regulators of Rho GTPase activity in development and disease. Oncogene (2013) 1.44
Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G⁹³A transgenic ALS mice. J Neurosci (2011) 1.29
Amyotrophic lateral sclerosis 2-deficiency leads to neuronal degeneration in amyotrophic lateral sclerosis through altered AMPA receptor trafficking. J Neurosci (2006) 1.13
In Vivo imaging reveals distinct inflammatory activity of CNS microglia versus PNS macrophages in a mouse model for ALS. PLoS One (2011) 1.10
Fishing for causes and cures of motor neuron disorders. Dis Model Mech (2014) 0.90
Influence of methylene blue on microglia-induced inflammation and motor neuron degeneration in the SOD1(G93A) model for ALS. PLoS One (2012) 0.89
Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis. Mol Neurodegener (2009) 0.89
Genetic rodent models of amyotrophic lateral sclerosis. J Biomed Biotechnol (2011) 0.87
Transgenic animal models of neurodegeneration based on human genetic studies. J Neural Transm (Vienna) (2010) 0.85
Aberrant neuregulin 1 signaling in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (2012) 0.85
Alsin and the molecular pathways of amyotrophic lateral sclerosis. Mol Neurobiol (2007) 0.83
ALS2/alsin knockout mice and motor neuron diseases. Neurodegener Dis (2008) 0.81
Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function. J Neuroimmunol (2006) 0.77
Absence of alsin function leads to corticospinal motor neuron vulnerability via novel disease mechanisms. Hum Mol Genet (2016) 0.77
An interrupted beta-propeller and protein disorder: structural bioinformatics insights into the N-terminus of alsin. J Mol Model (2008) 0.77
A Case for Microtubule Vulnerability in Amyotrophic Lateral Sclerosis: Altered Dynamics During Disease. Front Cell Neurosci (2016) 0.75
Onset and progression in inherited ALS determined by motor neurons and microglia. Science (2006) 9.42
ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron (2006) 7.82
On the road to cancer: aneuploidy and the mitotic checkpoint. Nat Rev Cancer (2005) 7.53
Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci (2004) 6.99
The human CENP-A centromeric nucleosome-associated complex. Nat Cell Biol (2006) 6.85
Boveri revisited: chromosomal instability, aneuploidy and tumorigenesis. Nat Rev Mol Cell Biol (2009) 6.43
Aneuploidy acts both oncogenically and as a tumor suppressor. Cancer Cell (2006) 6.37
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci (2008) 5.89
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol (2009) 5.65
Rethinking ALS: the FUS about TDP-43. Cell (2009) 4.71
Propagation of centromeric chromatin requires exit from mitosis. J Cell Biol (2007) 4.60
Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron (2012) 4.48
A standardized kinesin nomenclature. J Cell Biol (2004) 4.43
Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A (2002) 4.37
Structural determinants for generating centromeric chromatin. Nature (2004) 4.25
Centromere-specific assembly of CENP-a nucleosomes is mediated by HJURP. Cell (2009) 4.22
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2009) 4.18
Does aneuploidy cause cancer? Curr Opin Cell Biol (2006) 3.89
ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci (2008) 3.78
Decoding the links between mitosis, cancer, and chemotherapy: The mitotic checkpoint, adaptation, and cell death. Cancer Cell (2005) 3.69
Lethality to human cancer cells through massive chromosome loss by inhibition of the mitotic checkpoint. Proc Natl Acad Sci U S A (2004) 3.69
Dynamics of centromere and kinetochore proteins; implications for checkpoint signaling and silencing. Curr Biol (2004) 3.65
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Unattached kinetochores catalyze production of an anaphase inhibitor that requires a Mad2 template to prime Cdc20 for BubR1 binding. Dev Cell (2009) 3.36
Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron (2002) 3.31
Unstable kinetochore-microtubule capture and chromosomal instability following deletion of CENP-E. Dev Cell (2002) 3.23
Centromere-associated protein-E is essential for the mammalian mitotic checkpoint to prevent aneuploidy due to single chromosome loss. J Cell Biol (2003) 3.15
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat Neurosci (2002) 3.09
Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease. Nat Neurosci (2007) 3.02
Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest (2006) 3.00
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
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 2.94
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci (2012) 2.93
Delayed diagnosis in duchenne muscular dystrophy: data from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet). J Pediatr (2009) 2.90
Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell (2012) 2.83
Centromere identity maintained by nucleosomes assembled with histone H3 containing the CENP-A targeting domain. Mol Cell (2007) 2.76
Epigenetic centromere propagation and the nature of CENP-a nucleosomes. Cell (2011) 2.74
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci (2013) 2.71
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
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol (2011) 2.48
Activating and silencing the mitotic checkpoint through CENP-E-dependent activation/inactivation of BubR1. Cell (2003) 2.47
Cep152 interacts with Plk4 and is required for centriole duplication. J Cell Biol (2010) 2.45
ZW10 links mitotic checkpoint signaling to the structural kinetochore. J Cell Biol (2005) 2.36
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A (2008) 2.35
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia. Proc Natl Acad Sci U S A (2013) 2.31
Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2007) 2.25
An epigenetic mark generated by the incorporation of CENP-A into centromeric nucleosomes. Proc Natl Acad Sci U S A (2007) 2.23
An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med (2007) 2.20
Aneuploidy: instigator and inhibitor of tumorigenesis. Cancer Res (2007) 2.12
Microtubule capture by CENP-E silences BubR1-dependent mitotic checkpoint signaling. J Cell Biol (2005) 2.11
Catalytic assembly of the mitotic checkpoint inhibitor BubR1-Cdc20 by a Mad2-induced functional switch in Cdc20. Mol Cell (2013) 2.06
Polo-like kinase 4 kinase activity limits centrosome overduplication by autoregulating its own stability. J Cell Biol (2010) 2.05
The seeds of neurodegeneration: prion-like spreading in ALS. Cell (2011) 2.03
Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol (2003) 2.02
Aurora kinases and protein phosphatase 1 mediate chromosome congression through regulation of CENP-E. Cell (2010) 2.00
Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2006) 1.97
CENP-A-containing nucleosomes: easier disassembly versus exclusive centromeric localization. J Mol Biol (2007) 1.93
Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab (2012) 1.92
Heparan sulfate proteoglycans mediate internalization and propagation of specific proteopathic seeds. Proc Natl Acad Sci U S A (2013) 1.88
Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J Clin Invest (2009) 1.86
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A (2008) 1.84
Losing balance: the origin and impact of aneuploidy in cancer. EMBO Rep (2012) 1.81
Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A (2007) 1.79
CENP-E combines a slow, processive motor and a flexible coiled coil to produce an essential motile kinetochore tether. J Cell Biol (2008) 1.77
Removal of Spindly from microtubule-attached kinetochores controls spindle checkpoint silencing in human cells. Genes Dev (2010) 1.66
Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron (2010) 1.65
A two-step mechanism for epigenetic specification of centromere identity and function. Nat Cell Biol (2013) 1.52
Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci (2013) 1.51
Medicine. Treating neurodegenerative diseases with antibiotics. Science (2005) 1.51
Prion-like spread of protein aggregates in neurodegeneration. J Exp Med (2012) 1.49
Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F. EMBO J (2005) 1.48
NuMA after 30 years: the matrix revisited. Trends Cell Biol (2010) 1.48
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A (2013) 1.48
Double-strand DNA breaks recruit the centromeric histone CENP-A. Proc Natl Acad Sci U S A (2009) 1.47
Requirements for NuMA in maintenance and establishment of mammalian spindle poles. J Cell Biol (2009) 1.47
Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A (2008) 1.45
Human Zwint-1 specifies localization of Zeste White 10 to kinetochores and is essential for mitotic checkpoint signaling. J Biol Chem (2004) 1.44
Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann Neurol (2005) 1.44
Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A (2013) 1.44
Chromosomal instability by inefficient Mps1 auto-activation due to a weakened mitotic checkpoint and lagging chromosomes. PLoS One (2008) 1.43
Misregulated RNA processing in amyotrophic lateral sclerosis. Brain Res (2012) 1.42
NF-M is an essential target for the myelin-directed "outside-in" signaling cascade that mediates radial axonal growth. J Cell Biol (2003) 1.41
The role of aneuploidy in promoting and suppressing tumors. J Cell Biol (2009) 1.39
Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS. Mol Ther (2013) 1.36
Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS One (2008) 1.34
Inducible, reversible system for the rapid and complete degradation of proteins in mammalian cells. Proc Natl Acad Sci U S A (2012) 1.33
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A (2009) 1.33