Published in Hum Mol Genet on August 31, 2010
Exome sequencing identifies a DYNC1H1 mutation in a large pedigree with dominant axonal Charcot-Marie-Tooth disease. Am J Hum Genet (2011) 1.77
Cytoplasmic dynein heavy chain: the servant of many masters. Trends Neurosci (2013) 1.09
Huntington's disease and the striatal medium spiny neuron: cell-autonomous and non-cell-autonomous mechanisms of disease. Neurotherapeutics (2012) 0.98
Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age. Neurobiol Dis (2013) 0.96
Neuromuscular junction defects in mice with mutation of dynein heavy chain 1. PLoS One (2011) 0.91
Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization. Ann Neurol (2014) 0.90
Genetic rodent models of amyotrophic lateral sclerosis. J Biomed Biotechnol (2011) 0.87
Alteration of dynein function affects α-synuclein degradation via the autophagosome-lysosome pathway. Int J Mol Sci (2013) 0.82
PATHOLOGIES OF AXONAL TRANSPORT IN NEURODEGENERATIVE DISEASES. Transl Neurosci (2012) 0.81
Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice. Antioxid Redox Signal (2015) 0.81
A novel phenotype for the dynein heavy chain mutation Loa: altered dendritic morphology, organelle density, and reduced numbers of trigeminal motoneurons. J Comp Neurol (2012) 0.80
Altered hypothalamic protein expression in a rat model of Huntington's disease. PLoS One (2012) 0.79
Diffusion tensor magnetic resonance imaging of the brain in APP transgenic mice: a cohort study. PLoS One (2013) 0.78
Low dietary protein content alleviates motor symptoms in mice with mutant dynactin/dynein-mediated neurodegeneration. Hum Mol Genet (2014) 0.77
Mice with mutation in dynein heavy chain 1 do not share the same tau expression pattern with mice with SOD1-related motor neuron disease. Neurochem Res (2011) 0.77
A systems approach identifies networks and genes linking sleep and stress: implications for neuropsychiatric disorders. Cell Rep (2015) 0.77
Dynein at odd angles? Nat Cell Biol (2010) 0.75
Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases. Neurobiol Dis (2017) 0.75
Unbiased stereological estimation of the total number of neurons in thesubdivisions of the rat hippocampus using the optical fractionator. Anat Rec (1991) 11.44
Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice. Cell (2004) 8.58
Simplified reference tissue model for PET receptor studies. Neuroimage (1996) 8.28
Dynactin increases the processivity of the cytoplasmic dynein motor. Nat Cell Biol (2000) 6.13
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell (2004) 5.67
Mutant dynactin in motor neuron disease. Nat Genet (2003) 5.28
Mutations in dynein link motor neuron degeneration to defects in retrograde transport. Science (2003) 4.40
Cytoplasmic dynein-dependent vesicular transport from early to late endosomes. J Cell Biol (1993) 4.12
DCTN1 mutations in Perry syndrome. Nat Genet (2009) 3.87
Dynein mutations impair autophagic clearance of aggregate-prone proteins. Nat Genet (2005) 3.39
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab (2006) 3.38
Branching out: mechanisms of dendritic arborization. Nat Rev Neurosci (2010) 3.26
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration. Neuron (2002) 3.08
Axonal transport and neurodegenerative disease. Biochim Biophys Acta (2006) 3.06
Drosophila Lis1 is required for neuroblast proliferation, dendritic elaboration and axonal transport. Nat Cell Biol (2000) 3.05
Imaging synaptic neurotransmission with in vivo binding competition techniques: a critical review. J Cereb Blood Flow Metab (2000) 2.95
Axonal transport defects in neurodegenerative diseases. J Neurosci (2009) 2.74
A huntingtin-associated protein enriched in brain with implications for pathology. Nature (1995) 2.67
Distal spinal and bulbar muscular atrophy caused by dynactin mutation. Ann Neurol (2005) 2.34
Dynein is required for polarized dendritic transport and uniform microtubule orientation in axons. Nat Cell Biol (2008) 2.33
Dichotomous anatomical properties of adult striatal medium spiny neurons. J Neurosci (2008) 2.29
Proprioceptive sensory neuropathy in mice with a mutation in the cytoplasmic Dynein heavy chain 1 gene. J Neurosci (2007) 2.23
Huntingtin facilitates dynein/dynactin-mediated vesicle transport. Proc Natl Acad Sci U S A (2007) 2.16
Early striatal dendrite deficits followed by neuron loss with advanced age in the absence of anterograde cortical brain-derived neurotrophic factor. J Neurosci (2004) 2.16
Point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS. Neurology (2004) 2.14
Polarized signaling endosomes coordinate BDNF-induced chemotaxis of cerebellar precursors. Neuron (2007) 2.13
A motor neuron disease-associated mutation in p150Glued perturbs dynactin function and induces protein aggregation. J Cell Biol (2006) 2.08
Spatial control of branching within dendritic arbors by dynein-dependent transport of Rab5-endosomes. Nat Cell Biol (2008) 2.04
A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J Cell Biol (2005) 2.01
Interaction of huntingtin-associated protein with dynactin P150Glued. J Neurosci (1998) 1.98
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin. Nat Neurosci (2009) 1.95
A switch in retrograde signaling from survival to stress in rapid-onset neurodegeneration. J Neurosci (2009) 1.90
Global deprivation of brain-derived neurotrophic factor in the CNS reveals an area-specific requirement for dendritic growth. J Neurosci (2010) 1.85
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. Nat Genet (1998) 1.83
Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. J Neurosci (2002) 1.48
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
Motor neuron disease occurring in a mutant dynactin mouse model is characterized by defects in vesicular trafficking. J Neurosci (2008) 1.45
Roles of the cytoskeleton and motor proteins in endocytic sorting. Adv Drug Deliv Rev (2003) 1.36
The G59S mutation in p150(glued) causes dysfunction of dynactin in mice. J Neurosci (2007) 1.36
Lysosomal proliferation and distal degeneration in motor neurons expressing the G59S mutation in the p150Glued subunit of dynactin. Hum Mol Genet (2008) 1.31
A dynein mutation attenuates motor neuron degeneration in SOD1(G93A) mice. Exp Neurol (2006) 1.23
Hippocampal neurons recycle BDNF for activity-dependent secretion and LTP maintenance. EMBO J (2006) 1.22
Hippocampal abnormalities and enhanced excitability in a murine model of human lissencephaly. J Neurosci (2000) 1.20
SPM-based count normalization provides excellent discrimination of mild Alzheimer's disease and amnestic mild cognitive impairment from healthy aging. Neuroimage (2008) 1.16
Parametric mapping of binding in human brain of D2 receptor ligands of different affinities. J Nucl Med (2005) 1.15
Dynactin enhances the processivity of kinesin-2. Traffic (2006) 1.12
Elucidating the genetics and pathology of Perry syndrome. J Neurol Sci (2009) 1.12
Cytoplasmic dynein/dynactin function and dysfunction in motor neurons. Int J Dev Neurosci (2006) 1.11
A novel mouse model with impaired dynein/dynactin function develops amyotrophic lateral sclerosis (ALS)-like features in motor neurons and improves lifespan in SOD1-ALS mice. Hum Mol Genet (2008) 1.09
Ablation of D1 dopamine receptor-expressing cells generates mice with seizures, dystonia, hyperactivity, and impaired oral behavior. Proc Natl Acad Sci U S A (2007) 1.09
Hereditary sensory neuropathy type I. Orphanet J Rare Dis (2008) 1.08
Stimulated nuclear translocation of NF-kappaB and shuttling differentially depend on dynein and the dynactin complex. Proc Natl Acad Sci U S A (2009) 1.08
Sex differences in a transgenic rat model of Huntington's disease: decreased 17beta-estradiol levels correlate with reduced numbers of DARPP32+ neurons in males. Hum Mol Genet (2008) 1.07
G protein-coupled receptor kinase-2 constitutively regulates D2 dopamine receptor expression and signaling independently of receptor phosphorylation. J Biol Chem (2009) 1.06
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease. Exp Neurol (2008) 1.05
Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease. Mov Disord (2002) 1.00
AKT and CDK5/p35 mediate brain-derived neurotrophic factor induction of DARPP-32 in medium size spiny neurons in vitro. J Biol Chem (2007) 0.99
Neural cell adhesion molecule modulates dopaminergic signaling and behavior by regulating dopamine D2 receptor internalization. J Neurosci (2009) 0.98
Evaluation of R6/2 HD transgenic mice for therapeutic studies in Huntington's disease: behavioral testing and impact of diabetes mellitus. Behav Brain Res (2001) 0.97
Early exploratory behavior abnormalities in R6/1 Huntington's disease transgenic mice. Brain Res (2004) 0.92
Short-term striatal gene expression responses to brain-derived neurotrophic factor are dependent on MEK and ERK activation. PLoS One (2009) 0.89
Signaling by dopamine regulates D2 receptors trafficking at the membrane. Cell Cycle (2008) 0.88
D2 dopamine receptor expression and trafficking is regulated through direct interactions with ZIP. J Neurochem (2008) 0.85
p150/glued modifies nuclear estrogen receptor function. Mol Endocrinol (2009) 0.80
Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet (2009) 4.38
Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes. Ann Neurol (2007) 2.37
Rotigotine effects on early morning motor function and sleep in Parkinson's disease: a double-blind, randomized, placebo-controlled study (RECOVER). Mov Disord (2010) 2.34
Towards quantitative PET/MRI: a review of MR-based attenuation correction techniques. Eur J Nucl Med Mol Imaging (2009) 2.28
Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model. Proc Natl Acad Sci U S A (2004) 2.26
X-linked bulbospinal neuronopathy: Kennedy disease. Arch Neurol (2002) 2.21
Guidelines for preclinical animal research in ALS/MND: A consensus meeting. Amyotroph Lateral Scler (2010) 2.14
Are amygdalar volume alterations in children with Tourette syndrome due to ADHD comorbidity? Dev Med Child Neurol (2008) 2.11
Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol (2010) 2.00
TNFR1 signaling and IFN-gamma signaling determine whether T cells induce tumor dormancy or promote multistage carcinogenesis. Cancer Cell (2008) 1.91
Critical loss of CBP/p300 histone acetylase activity by caspase-6 during neurodegeneration. EMBO J (2003) 1.80
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol (2008) 1.78
REEP1 mutation spectrum and genotype/phenotype correlation in hereditary spastic paraplegia type 31. Brain (2008) 1.77
Four familial ALS pedigrees discordant for two SOD1 mutations: are all SOD1 mutations pathogenic? J Neurol Neurosurg Psychiatry (2010) 1.68
Transgenic rat model of Huntington's disease. Hum Mol Genet (2003) 1.66
Nogo-A, -B, and -C are found on the cell surface and interact together in many different cell types. J Biol Chem (2005) 1.61
Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel-based morphometry of 3-D MRI. Amyotroph Lateral Scler Other Motor Neuron Disord (2005) 1.55
Sodium valproate exerts neuroprotective effects in vivo through CREB-binding protein-dependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model. J Neurosci (2007) 1.52
Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models. Curr Opin Pharmacol (2009) 1.47
Images in cardiovascular medicine. Cardiomyopathy in a Duchenne muscular dystrophy carrier and her diseased son: similar pattern revealed by cardiovascular MRI. Circulation (2010) 1.47
Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol (2011) 1.45
Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons. PLoS One (2009) 1.45
Cell tracking with gadophrin-2: a bifunctional contrast agent for MR imaging, optical imaging, and fluorescence microscopy. Eur J Nucl Med Mol Imaging (2004) 1.43
Heterozygous R1101K mutation of the DCTN1 gene in a family with ALS and FTD. Ann Neurol (2005) 1.42
Towards a neuroimaging biomarker for amyotrophic lateral sclerosis. Lancet Neurol (2011) 1.40
Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabil Neural Repair (2005) 1.40
Selective E2F-dependent gene transcription is controlled by histone deacetylase activity during neuronal apoptosis. J Neurochem (2003) 1.37
SOX2 expression associates with stem cell state in human ovarian carcinoma. Cancer Res (2013) 1.34
The chemokine CXCL13 is a prognostic marker in clinically isolated syndrome (CIS). PLoS One (2010) 1.32
Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study. J Neurol Sci (2005) 1.31
Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model. Physiol Genomics (2007) 1.31
HP1alpha guides neuronal fate by timing E2F-targeted genes silencing during terminal differentiation. EMBO J (2007) 1.31
Amyotrophic lateral sclerosis--a model of corticofugal axonal spread. Nat Rev Neurol (2013) 1.29
The chemokine CXCL13 in acute neuroborreliosis. J Neurol Neurosurg Psychiatry (2009) 1.26
Cytoplasmic dynein in neurodegeneration. Pharmacol Ther (2011) 1.25
IgG antibodies against measles, rubella, and varicella zoster virus predict conversion to multiple sclerosis in clinically isolated syndrome. PLoS One (2009) 1.25
Daptomycin is highly efficacious against penicillin-resistant and penicillin- and quinolone-resistant pneumococci in experimental meningitis. Antimicrob Agents Chemother (2004) 1.23
Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging. Hum Brain Mapp (2010) 1.23
Targeting CREB-binding protein (CBP) loss of function as a therapeutic strategy in neurological disorders. Biochem Pharmacol (2004) 1.22
Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol (2007) 1.21
Spatial memory consolidation is associated with induction of several lysine-acetyltransferase (histone acetyltransferase) expression levels and H2B/H4 acetylation-dependent transcriptional events in the rat hippocampus. Neuropsychopharmacology (2010) 1.21
Impaired glucose tolerance in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2010) 1.20
A new missense mutation in the leptin gene causes mild obesity and hypogonadism without affecting T cell responsiveness. J Clin Endocrinol Metab (2010) 1.19
Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis. Neurobiol Dis (2002) 1.17
The cerebral control of speech tempo: opposite relationship between speaking rate and BOLD signal changes at striatal and cerebellar structures. Neuroimage (2005) 1.17
Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.16
Brain morphology alterations in the basal ganglia and the hypothalamus following prenatal exposure to antiepileptic drugs. Eur J Paediatr Neurol (2007) 1.15
Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS--Parkinson Plus Scale. PLoS One (2011) 1.15
Alternative trial design in amyotrophic lateral sclerosis saves time and patients. Amyotroph Lateral Scler (2007) 1.15
Mutant huntingtin causes metabolic imbalance by disruption of hypothalamic neurocircuits. Cell Metab (2011) 1.14
18F-labeling of peptides by means of an organosilicon-based fluoride acceptor. Angew Chem Int Ed Engl (2006) 1.13
Enhanced visualization of blurred gray-white matter junctions in focal cortical dysplasia by voxel-based 3D MRI analysis. Epilepsy Res (2005) 1.13
Activation of microglial N-methyl-D-aspartate receptors triggers inflammation and neuronal cell death in the developing and mature brain. Ann Neurol (2012) 1.12
The modulation of Amyotrophic Lateral Sclerosis risk by ataxin-2 intermediate polyglutamine expansions is a specific effect. Neurobiol Dis (2011) 1.12
Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis. FASEB J (2003) 1.11
Nogo expression in muscle correlates with amyotrophic lateral sclerosis severity. Ann Neurol (2005) 1.11
Neuroectodermally converted human mesenchymal stromal cells provide cytoprotective effects on neural stem cells and inhibit their glial differentiation. Cytotherapy (2010) 1.10
Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis. J Lipid Res (2007) 1.09
Accelerated aging phenotype in mice with conditional deficiency for mitochondrial superoxide dismutase in the connective tissue. Aging Cell (2010) 1.09
The neurite outgrowth inhibitor Nogo-A promotes denervation in an amyotrophic lateral sclerosis model. EMBO Rep (2006) 1.09
A randomized, double blind, placebo-controlled trial of pioglitazone in combination with riluzole in amyotrophic lateral sclerosis. PLoS One (2012) 1.09
Increase in BDNF-mediated TrkB signaling promotes epileptogenesis in a mouse model of mesial temporal lobe epilepsy. Neurobiol Dis (2011) 1.08
Transdermal rotigotine for the perioperative management of Parkinson's disease. J Neural Transm (Vienna) (2010) 1.08
CNS-targeted viral delivery of G-CSF in an animal model for ALS: improved efficacy and preservation of the neuromuscular unit. Mol Ther (2010) 1.08
Histopathological analysis of skeletal muscle in patients with Parkinson's disease and 'dropped head'/'bent spine' syndrome. Parkinsonism Relat Disord (2009) 1.08
Mitochondria in amyotrophic lateral sclerosis: a trigger and a target. Neurodegener Dis (2004) 1.07
Spatial imagery in deductive reasoning: a functional MRI study. Brain Res Cogn Brain Res (2002) 1.07
NF-κB is required for Smac mimetic-mediated sensitization of glioblastoma cells for γ-irradiation-induced apoptosis. Mol Cancer Ther (2011) 1.06
Late-onset motoneuron disease caused by a functionally modified AMPA receptor subunit. Proc Natl Acad Sci U S A (2005) 1.06
A ruthenium-containing organometallic compound reduces tumor growth through induction of the endoplasmic reticulum stress gene CHOP. Cancer Res (2009) 1.06
Linking neuron and skin: matrix metalloproteinases in amyotrophic lateral sclerosis (ALS). J Neurol Sci (2009) 1.05
Cyclin-dependent kinases phosphorylate p73 at threonine 86 in a cell cycle-dependent manner and negatively regulate p73. J Biol Chem (2003) 1.05
Phosphoinositide 3-kinases upregulate system xc(-) via eukaryotic initiation factor 2α and activating transcription factor 4 - A pathway active in glioblastomas and epilepsy. Antioxid Redox Signal (2014) 1.05
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease. Exp Neurol (2008) 1.05
Enhancement of p53 activity and inhibition of neural cell proliferation by glucocorticoid receptor activation. FASEB J (2002) 1.04