Published in Proc Natl Acad Sci U S A on April 26, 2007
Amyotrophic Lateral Sclerosis and the Innate Immune System | NCT02869048
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat Neurosci (2009) 3.89
ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci (2008) 3.78
Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol (2011) 3.45
The changing scene of amyotrophic lateral sclerosis. Nat Rev Neurosci (2013) 3.16
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol (2011) 2.73
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
Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase. Proc Natl Acad Sci U S A (2010) 1.60
Progranulin is expressed within motor neurons and promotes neuronal cell survival. BMC Neurosci (2009) 1.46
Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy. Proc Natl Acad Sci U S A (2013) 1.35
The unfolded protein response in familial amyotrophic lateral sclerosis. Hum Mol Genet (2010) 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
C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A (2013) 1.17
D-amino acid oxidase controls motoneuron degeneration through D-serine. Proc Natl Acad Sci U S A (2011) 1.10
Reduced activity of AMP-activated protein kinase protects against genetic models of motor neuron disease. J Neurosci (2012) 1.06
The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis. Sci Rep (2012) 1.06
Activation of interferon signaling pathways in spinal cord astrocytes from an ALS mouse model. Glia (2011) 1.02
Vascular endothelial growth factor prevents G93A-SOD1-induced motor neuron degeneration. Dev Neurobiol (2009) 1.02
Improved RNA preservation for immunolabeling and laser microdissection. RNA (2009) 1.02
Versatility of the complement system in neuroinflammation, neurodegeneration and brain homeostasis. Front Cell Neurosci (2014) 1.01
Cellular therapy to target neuroinflammation in amyotrophic lateral sclerosis. Cell Mol Life Sci (2013) 1.00
Complement is dispensable for neurodegeneration in Niemann-Pick disease type C. J Neuroinflammation (2012) 1.00
Spinal cord trauma and the molecular point of no return. Mol Neurodegener (2012) 1.00
An enhanced integrated stress response ameliorates mutant SOD1-induced ALS. Hum Mol Genet (2013) 0.98
RNA-Seq profiling of spinal cord motor neurons from a presymptomatic SOD1 ALS mouse. PLoS One (2013) 0.98
Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet (2013) 0.96
Motor neuron-immune interactions: the vicious circle of ALS. J Neural Transm (Vienna) (2010) 0.93
Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS. Proc Natl Acad Sci U S A (2015) 0.93
Laser capture microscopy coupled with Smart-seq2 for precise spatial transcriptomic profiling. Nat Commun (2016) 0.86
System xC- is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice. Brain (2014) 0.86
Identification of pharmacological targets in amyotrophic lateral sclerosis through genomic analysis of deregulated genes and pathways. Curr Genomics (2012) 0.84
Mechanisms of granulin deficiency: lessons from cellular and animal models. Mol Neurobiol (2012) 0.84
Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis. Brain (2015) 0.83
The importance of molecular histology to study glial influence on neurodegenerative disorders. Focus on recent developed single cell laser microdissection. J Mol Histol (2009) 0.82
Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2015) 0.81
ATF3 expression improves motor function in the ALS mouse model by promoting motor neuron survival and retaining muscle innervation. Proc Natl Acad Sci U S A (2014) 0.81
Axotomy-induced target disconnection promotes an additional death mechanism involved in motoneuron degeneration in amyotrophic lateral sclerosis transgenic mice. J Comp Neurol (2014) 0.80
Metabolomic Analysis Reveals Increased Aerobic Glycolysis and Amino Acid Deficit in a Cellular Model of Amyotrophic Lateral Sclerosis. Mol Neurobiol (2015) 0.79
Deregulated expression of cytoskeleton related genes in the spinal cord and sciatic nerve of presymptomatic SOD1(G93A) Amyotrophic Lateral Sclerosis mouse model. Front Cell Neurosci (2014) 0.79
Complement activation at the motor end-plates in amyotrophic lateral sclerosis. J Neuroinflammation (2016) 0.77
Current and future directions in genomics of amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am (2008) 0.77
Stathmin 1/2-triggered microtubule loss mediates Golgi fragmentation in mutant SOD1 motor neurons. Mol Neurodegener (2016) 0.75
Production of RNA for transcriptomic analysis from mouse spinal cord motor neuron cell bodies by laser capture microdissection. J Vis Exp (2014) 0.75
Analysis of monocyte infiltration in MPTP mice reveals that microglial CX3CR1 protects against neurotoxic over-induction of monocyte-attracting CCL2 by astrocytes. J Neuroinflammation (2017) 0.75
An indicator cell assay for blood-based diagnostics. PLoS One (2017) 0.75
Model-based analysis of oligonucleotide arrays: expression index computation and outlier detection. Proc Natl Acad Sci U S A (2001) 68.44
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature (1993) 21.65
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science (1994) 18.08
Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature (2006) 11.59
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
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science (2003) 6.35
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron (1997) 6.16
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron (1995) 6.03
Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science (1998) 5.23
Glia-derived D-serine controls NMDA receptor activity and synaptic memory. Cell (2006) 4.53
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
Activating transcription factor 3 (ATF3) induction by axotomy in sensory and motoneurons: A novel neuronal marker of nerve injury. Mol Cell Neurosci (2000) 3.73
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci (2005) 3.69
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. Nat Neurosci (2006) 3.56
Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity. Proc Natl Acad Sci U S A (1994) 3.36
The AP-1 transcription factor c-Jun is required for efficient axonal regeneration. Neuron (2004) 3.33
Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron (2002) 3.31
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron (2004) 2.94
Small proline-rich repeat protein 1A is expressed by axotomized neurons and promotes axonal outgrowth. J Neurosci (2002) 2.01
Overexpression of SOD1 in transgenic rats protects vulnerable neurons against ischemic damage after global cerebral ischemia and reperfusion. J Neurosci (1998) 1.99
Neuron-derived D-serine release provides a novel means to activate N-methyl-D-aspartate receptors. J Biol Chem (2006) 1.95
C1q: structure, function, and receptors. Immunopharmacology (2000) 1.89
Role of complement in neurodegeneration and neuroinflammation. Mol Immunol (2006) 1.73
Absence of C1q leads to less neuropathology in transgenic mouse models of Alzheimer's disease. J Neurosci (2004) 1.66
Neuronal protection in stroke by an sLex-glycosylated complement inhibitory protein. Science (1999) 1.65
Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem (2002) 1.63
CXCR3-dependent microglial recruitment is essential for dendrite loss after brain lesion. J Neurosci (2004) 1.57
Natural history and inherited disorders of a lysosomal enzyme, beta-hexosaminidase. J Biol Chem (1989) 1.48
Localization and cell association of C1q in Alzheimer's disease brain. Exp Neurol (1996) 1.32
Neuronal expression of mRNAs for complement proteins of the classical pathway in Alzheimer brain. Brain Res (1997) 1.24
Chronic activation in presymptomatic amyotrophic lateral sclerosis (ALS) mice of a feedback loop involving Fas, Daxx, and FasL. Proc Natl Acad Sci U S A (2006) 1.23
Cardiotrophin-1, a cytokine present in embryonic muscle, supports long-term survival of spinal motoneurons. Neuron (1996) 1.19
Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A (2005) 1.19
Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis. Neurosci Lett (2003) 1.17
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem (2005) 1.16
Neurons express proteins of the classical complement pathway in Alzheimer disease. Brain Res (1997) 1.15
Disease mechanisms revealed by transcription profiling in SOD1-G93A transgenic mouse spinal cord. Ann Neurol (2001) 1.10
ATF3 expression precedes death of spinal motoneurons in amyotrophic lateral sclerosis-SOD1 transgenic mice and correlates with c-Jun phosphorylation, CHOP expression, somato-dendritic ubiquitination and Golgi fragmentation. Eur J Neurosci (2005) 1.07
Deposits of IgG and C3 in the spinal cord and motor cortex of ALS patients. J Neuroimmunol (1984) 1.02
No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model. Hum Mol Genet (2005) 1.01
Cu/Zn-superoxide dismutase (GLY93-->ALA) mutation alters AMPA receptor subunit expression and function and potentiates kainate-mediated toxicity in motor neurons in culture. Neurobiol Dis (2004) 0.96
Complement C3c and related protein biomarkers in amyotrophic lateral sclerosis and Parkinson's disease. Biochem Biophys Res Commun (2006) 0.93
Neuronal localization of C1q in preclinical Alzheimer's disease. Neurobiol Dis (2004) 0.92
C1qB and clusterin mRNA increase in association with neurodegeneration in sporadic amyotrophic lateral sclerosis. Neurosci Lett (1999) 0.88
Novel neuroglial and glioglial relationships mediated by L-serine metabolism. Arch Histol Cytol (2003) 0.86
Differential regulation of Abeta42-induced neuronal C1q synthesis and microglial activation. J Neuroinflammation (2005) 0.85
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
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
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
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
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
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
Chromoanagenesis and cancer: mechanisms and consequences of localized, complex chromosomal rearrangements. Nat Med (2012) 1.32
Chromosome missegregation rate predicts whether aneuploidy will promote or suppress tumors. Proc Natl Acad Sci U S A (2013) 1.28
Human condensin function is essential for centromeric chromatin assembly and proper sister kinetochore orientation. PLoS One (2009) 1.27
ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A (2010) 1.26
A chemical tool box defines mitotic and interphase roles for Mps1 kinase. J Cell Biol (2010) 1.25
The autoregulated instability of Polo-like kinase 4 limits centrosome duplication to once per cell cycle. Genes Dev (2012) 1.23
Error-prone mammalian female meiosis from silencing the spindle assembly checkpoint without normal interkinetochore tension. Proc Natl Acad Sci U S A (2012) 1.23
The aneuploidy paradox in cell growth and tumorigenesis. Cancer Cell (2008) 1.19
Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A (2005) 1.19
Phenotype difference between ALS patients with expanded repeats in C9ORF72 and patients with mutations in other ALS-related genes. J Med Genet (2012) 1.19
Modest loss of peripheral axons, muscle atrophy and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1. J Neurochem (2008) 1.18
C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A (2013) 1.17
Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology. Acta Neuropathol (2013) 1.16
Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem (2005) 1.16
Hindlimb gait defects due to motor axon loss and reduced distal muscles in a transgenic mouse model of Charcot-Marie-Tooth type 2A. Hum Mol Genet (2007) 1.16
Cell biology: nondisjunction, aneuploidy and tetraploidy. Nature (2006) 1.15