Published in PLoS One on August 22, 2007
Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB mice. PLoS One (2008) 1.30
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The imprinted gene Magel2 regulates normal circadian output. Nat Genet (2007) 2.18
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Acquired deficit of forebrain glucocorticoid receptor produces depression-like changes in adrenal axis regulation and behavior. Proc Natl Acad Sci U S A (2004) 2.11
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Clock genes, oscillators, and cellular networks in the suprachiasmatic nuclei. J Biol Rhythms (2004) 2.07
Gene targeting in vivo by adeno-associated virus vectors. Nat Biotechnol (2006) 2.03
Brain fatty acid synthase activates PPARalpha to maintain energy homeostasis. J Clin Invest (2007) 2.03
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Treatment with an amyloid-beta antibody ameliorates plaque load, learning deficits, and hippocampal long-term potentiation in a mouse model of Alzheimer's disease. J Neurosci (2005) 1.90
Drug-induced apoptotic neurodegeneration in the developing brain. Brain Pathol (2002) 1.85
Sirt1 extends life span and delays aging in mice through the regulation of Nk2 homeobox 1 in the DMH and LH. Cell Metab (2013) 1.74
Intrathecal enzyme replacement therapy: successful treatment of brain disease via the cerebrospinal fluid. Mol Genet Metab (2007) 1.71
Intrinsic, nondeterministic circadian rhythm generation in identified mammalian neurons. Proc Natl Acad Sci U S A (2009) 1.70
Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis (2006) 1.69
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CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis. Mol Ther (2005) 1.66
GABA and Gi/o differentially control circadian rhythms and synchrony in clock neurons. Proc Natl Acad Sci U S A (2006) 1.62
Activity-dependent transcription regulation of PSD-95 by neuregulin-1 and Eos. Nat Neurosci (2004) 1.61
Circadian rhythm generation and entrainment in astrocytes. J Neurosci (2005) 1.59
Liver-directed neonatal gene therapy prevents cardiac, bone, ear, and eye disease in mucopolysaccharidosis I mice. Mol Ther (2005) 1.58
Circadian clock proteins regulate neuronal redox homeostasis and neurodegeneration. J Clin Invest (2013) 1.57
Forebrain glucocorticoid receptors modulate anxiety-associated locomotor activation and adrenal responsiveness. J Neurosci (2006) 1.56
In vivo distribution of human adipose-derived mesenchymal stem cells in novel xenotransplantation models. Stem Cells (2006) 1.56
Disrupted membrane homeostasis and accumulation of ubiquitinated proteins in a mouse model of infantile neuroaxonal dystrophy caused by PLA2G6 mutations. Am J Pathol (2008) 1.54
A molecular model for intercellular synchronization in the mammalian circadian clock. Biophys J (2007) 1.54
Exome-sequencing confirms DNAJC5 mutations as cause of adult neuronal ceroid-lipofuscinosis. PLoS One (2011) 1.51
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SIRT1 promotes the central adaptive response to diet restriction through activation of the dorsomedial and lateral nuclei of the hypothalamus. J Neurosci (2010) 1.51
Marrow stromal cells and osteoclast precursors differentially contribute to TNF-alpha-induced osteoclastogenesis in vivo. J Immunol (2004) 1.50
Chemically modified beta-glucuronidase crosses blood-brain barrier and clears neuronal storage in murine mucopolysaccharidosis VII. Proc Natl Acad Sci U S A (2008) 1.49
Inhibition of caspases prevents ototoxic and ongoing hair cell death. J Neurosci (2002) 1.46
Circadian regulation of ATP release in astrocytes. J Neurosci (2011) 1.41
Central amygdala glucocorticoid receptor action promotes fear-associated CRH activation and conditioning. Proc Natl Acad Sci U S A (2008) 1.41
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The suprachiasmatic nucleus entrains, but does not sustain, circadian rhythmicity in the olfactory bulb. J Neurosci (2004) 1.39
Abnormal glutamate homeostasis and impaired synaptic plasticity and learning in a mouse model of tuberous sclerosis complex. Neurobiol Dis (2007) 1.37
Increased soluble amyloid-beta peptide and memory deficits in amyloid model mice overexpressing the low-density lipoprotein receptor-related protein. Proc Natl Acad Sci U S A (2004) 1.36
Glycosylation-independent targeting enhances enzyme delivery to lysosomes and decreases storage in mucopolysaccharidosis type VII mice. Proc Natl Acad Sci U S A (2004) 1.33
Widespread nonhematopoietic tissue distribution by transplanted human progenitor cells with high aldehyde dehydrogenase activity. Stem Cells (2007) 1.30
Antisense reduction of tau in adult mice protects against seizures. J Neurosci (2013) 1.29
Cerebellar synaptic defects and abnormal motor behavior in mice lacking alpha- and beta-dystrobrevin. J Neurosci (2006) 1.28
Olfactory bulb neurons express functional, entrainable circadian rhythms. Eur J Neurosci (2004) 1.27
Low doses of memantine disrupt memory in adult rats. J Neurosci (2006) 1.27
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease. Hum Mol Genet (2009) 1.26
Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis. Neurobiol Dis (2004) 1.25
Expression of a truncated Sall1 transcriptional repressor is responsible for Townes-Brocks syndrome birth defects. Hum Mol Genet (2003) 1.25
A circadian clock in the olfactory bulb controls olfactory responsivity. J Neurosci (2006) 1.24
Independent circadian oscillations of Period1 in specific brain areas in vivo and in vitro. J Neurosci (2005) 1.22
AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2. Mol Ther (2005) 1.20
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A neuropeptide speeds circadian entrainment by reducing intercellular synchrony. Proc Natl Acad Sci U S A (2013) 1.19
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Critical role of beta3 integrin in experimental postmenopausal osteoporosis. J Bone Miner Res (2005) 1.17
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Lysosomal dysfunction results in altered energy balance. J Biol Chem (2007) 1.15
AAV2-mediated ocular gene therapy for infantile neuronal ceroid lipofuscinosis. Mol Ther (2005) 1.15
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Multiple episodes of mild traumatic brain injury result in impaired cognitive performance in mice. Acad Emerg Med (2004) 1.14
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Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse. Exp Neurol (2009) 1.13
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The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis. J Neurosci (2011) 1.12
Bone marrow transplantation augments the effect of brain- and spinal cord-directed adeno-associated virus 2/5 gene therapy by altering inflammation in the murine model of globoid-cell leukodystrophy. J Neurosci (2011) 1.12
GABA networks destabilize genetic oscillations in the circadian pacemaker. Neuron (2013) 1.12
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Small-world network models of intercellular coupling predict enhanced synchronization in the suprachiasmatic nucleus. J Biol Rhythms (2009) 1.11
Defining the pathway for Tat-mediated delivery of beta-glucuronidase in cultured cells and MPS VII mice. Mol Ther (2005) 1.10
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Mouse model of N-acetylgalactosamine-6-sulfate sulfatase deficiency (Galns-/-) produced by targeted disruption of the gene defective in Morquio A disease. Hum Mol Genet (2003) 1.08
Characterizing learning deficits and hippocampal neuron loss following transient global cerebral ischemia in rats. Brain Res (2005) 1.08