Published in J Neuropathol Exp Neurol on July 01, 2005
Neural stem cell gene therapy ameliorates pathology and function in a mouse model of globoid cell leukodystrophy. Stem Cells (2011) 1.22
Therapeutic benefit of lentiviral-mediated neonatal intracerebral gene therapy in a mouse model of globoid cell leukodystrophy. Hum Mol Genet (2014) 0.94
Galactocerebrosidase-deficient oligodendrocytes maintain stable central myelin by exogenous replacement of the missing enzyme in mice. Proc Natl Acad Sci U S A (2005) 0.88
Combined gene/cell therapies provide long-term and pervasive rescue of multiple pathological symptoms in a murine model of globoid cell leukodystrophy. Hum Mol Genet (2015) 0.87
MMP-3 mediates psychosine-induced globoid cell formation: implications for leukodystrophy pathology. Glia (2013) 0.85
Mechanism of neuromuscular dysfunction in Krabbe disease. J Neurosci (2015) 0.84
Cellular transplant therapies for globoid cell leukodystrophy: Preclinical and clinical observations. J Neurosci Res (2016) 0.75
Consequences of NPC1 and NPC2 loss of function in mammalian neurons. Biochim Biophys Acta (2004) 2.00
Functional interplay between RNA-binding protein HuR and microRNAs. Curr Protein Pept Sci (2012) 1.71
Plasma adiponectin levels in newborns are higher than those in adults and positively correlated with birth weight. Clin Endocrinol (Oxf) (2004) 1.69
Paranodal junction formation and spermatogenesis require sulfoglycolipids. Proc Natl Acad Sci U S A (2002) 1.68
Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease. Proc Natl Acad Sci U S A (2006) 1.62
Ghrelin concentration in cord and neonatal blood: relation to fetal growth and energy balance. J Clin Endocrinol Metab (2003) 1.56
A myelin galactolipid, sulfatide, is essential for maintenance of ion channels on myelinated axon but not essential for initial cluster formation. J Neurosci (2002) 1.51
Oligodendrocytes and progenitors become progressively depleted within chronically demyelinated lesions. Am J Pathol (2004) 1.35
Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease. Mol Genet Metab (2005) 1.32
Prostaglandin D2-mediated microglia/astrocyte interaction enhances astrogliosis and demyelination in twitcher. J Neurosci (2006) 1.32
Peripheral macrophage recruitment in cuprizone-induced CNS demyelination despite an intact blood-brain barrier. J Neuroimmunol (2002) 1.28
RNA-binding protein HuD controls insulin translation. Mol Cell (2012) 1.25
Translational control of TOP2A influences doxorubicin efficacy. Mol Cell Biol (2011) 1.25
NADPH oxidases in the gastrointestinal tract: a potential role of Nox1 in innate immune response and carcinogenesis. Antioxid Redox Signal (2006) 1.21
Global dissociation of HuR-mRNA complexes promotes cell survival after ionizing radiation. EMBO J (2011) 1.21
Enhanced translation by Nucleolin via G-rich elements in coding and non-coding regions of target mRNAs. Nucleic Acids Res (2011) 1.21
Functional genomic analysis of remyelination reveals importance of inflammation in oligodendrocyte regeneration. J Neurosci (2003) 1.18
Niemann-Pick type C disease: accelerated neurofibrillary tangle formation and amyloid beta deposition associated with apolipoprotein E epsilon 4 homozygosity. Ann Neurol (2002) 1.16
NADPH oxidase-derived reactive oxygen species are essential for differentiation of a mouse macrophage cell line (RAW264.7) into osteoclasts. J Med Invest (2009) 1.14
Unusual MR spectroscopic imaging pattern of an astrocytoma: lack of elevated choline and high myo-inositol and glycine levels. AJNR Am J Neuroradiol (2003) 1.13
Nox enzymes and oxidative stress in the immunopathology of the gastrointestinal tract. Semin Immunopathol (2008) 1.11
Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse. Hum Mol Genet (2004) 1.10
Interferon-gamma activates transcription of NADPH oxidase 1 gene and upregulates production of superoxide anion by human large intestinal epithelial cells. Am J Physiol Cell Physiol (2005) 1.07
Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse. J Neuropathol Exp Neurol (2004) 1.06
Post-Transcriptional Control of the Hypoxic Response by RNA-Binding Proteins and MicroRNAs. Front Mol Neurosci (2011) 1.06
Aberrant phosphorylation of alpha-synuclein in human Niemann-Pick type C1 disease. J Neuropathol Exp Neurol (2004) 1.03
The oncogenic RNA-binding protein Musashi1 is regulated by HuR via mRNA translation and stability in glioblastoma cells. Mol Cancer Res (2012) 1.02
Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region. Biochim Biophys Acta (2002) 0.98
Bezafibrate, a peroxisome proliferator-activated receptors agonist, decreases body temperature and enhances electroencephalogram delta-oscillation during sleep in mice. Endocrinology (2008) 0.97
Novel mutation and the first prenatal screening of cathepsin D deficiency (CLN10). Acta Neuropathol (2008) 0.96
Growth inhibition by miR-519 via multiple p21-inducing pathways. Mol Cell Biol (2012) 0.95
Perineuronal oligodendrocytes protect against neuronal apoptosis through the production of lipocalin-type prostaglandin D synthase in a genetic demyelinating model. J Neurosci (2002) 0.94
Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking. Hum Mol Genet (2007) 0.94
Receptor activator of nuclear factor-kappaB ligand-induced mouse osteoclast differentiation is associated with switching between NADPH oxidase homologues. Free Radic Biol Med (2009) 0.94
Anti-inflammatory therapy by ibudilast, a phosphodiesterase inhibitor, in demyelination of twitcher, a genetic demyelination model. J Neuroinflammation (2005) 0.92
The function of sphingolipids in the nervous system: lessons learnt from mouse models of specific sphingolipid activator protein deficiencies. J Neurochem (2007) 0.91
Tumor necrosis factor alpha activates transcription of the NADPH oxidase organizer 1 (NOXO1) gene and upregulates superoxide production in colon epithelial cells. Free Radic Biol Med (2008) 0.91
The protective role of nitric oxide in a neurotoxicant-induced demyelinating model. J Immunol (2002) 0.91
Niemann-Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles. Neurobiol Dis (2002) 0.91
Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides. J Neurochem (2006) 0.90
Effects of Bifidobacterium longum BB536 administration on influenza infection, influenza vaccine antibody titer, and cell-mediated immunity in the elderly. Biosci Biotechnol Biochem (2010) 0.88
MHC class II exacerbates demyelination in vivo independently of T cells. J Neuroimmunol (2008) 0.88
Hematopoietic prostaglandin D synthase is expressed in microglia in the developing postnatal mouse brain. Glia (2003) 0.87
Hematopoietic prostaglandin D synthase and DP1 receptor are selectively upregulated in microglia and astrocytes within senile plaques from human patients and in a mouse model of Alzheimer disease. J Neuropathol Exp Neurol (2007) 0.86
A de novo deafwaddler mutation of Pmca2 arising in ES cells and hitchhiking with a targeted modification of the Pparg gene. Mamm Genome (2006) 0.86
Fate of donor hematopoietic cells in demyelinating mutant mouse, twitcher, following transplantation of GFP+ bone marrow cells. Neurobiol Dis (2004) 0.86
Gene expression profiling in peripheral blood leukocytes as a new approach for assessment of human stress response. J Med Invest (2005) 0.86
Postmortem diagnosis of Fabry disease with acromegaly and a unique vasculopathy. Virchows Arch (2007) 0.85
Endosomal sorting by Semaphorin 4A in retinal pigment epithelium supports photoreceptor survival. Genes Dev (2012) 0.84
Peripheral neuropathy in the twitcher mouse: accumulation of extracellular matrix in the endoneurium and aberrant expression of ion channels. Acta Neuropathol (2008) 0.84
Developmental changes in glycolipids and synchronized expression of nutrient transporters in the mouse small intestine. J Nutr Biochem (2009) 0.82
A mutation in the saposin C domain of the sphingolipid activator protein (Prosaposin) gene causes neurodegenerative disease in mice. J Neurosci Res (2010) 0.81
Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice. J Lipid Res (2003) 0.80
Chemoenzymatic synthesis of hydrophobic glycoprotein: synthesis of saposin C carrying complex-type carbohydrate. J Org Chem (2012) 0.80
Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy. J Neurosci Res (2004) 0.80
Brief naturalistic stress induces an alternative splice variant of SMG-1 lacking exon 63 in peripheral leukocytes. Neurosci Lett (2010) 0.80
Successful allogeneic unrelated bone marrow transplantation using reduced-intensity conditioning for the treatment of X-linked adrenoleukodystrophy in a one-yr-old boy. Pediatr Transplant (2008) 0.78
Comparative clinico-pathological study of saposin-A-deficient (SAP-A-/-) and Twitcher mice. J Neuropathol Exp Neurol (2004) 0.78
Statistical analyses of Endogenous Uveitis Patients (2007-2009) in central Tokyo area and Comparison with Previous Studies (1963-2006). Ocul Immunol Inflamm (2014) 0.78
Improved high-fat diet-induced glucose intolerance by an oral administration of phytosphingosine. Biosci Biotechnol Biochem (2013) 0.77
High-throughput screening of brief naturalistic stress-responsive cytokines in university students taking examinations. Int J Psychophysiol (2010) 0.77
Regional expression of prosaposin in the wild-type and saposin D-deficient mouse brain detected by an anti-mouse prosaposin-specific antibody. Proc Jpn Acad Ser B Phys Biol Sci (2009) 0.76
Synthesis and crystal structure of a Pr5Ni19 superlattice alloy and its hydrogen absorption-desorption property. Inorg Chem (2011) 0.76
High-performance thin-layer chromatography/mass spectrometry for rapid analysis of neutral glycosphingolipids. J AOAC Int (2008) 0.75
High-performance thin-layer chromatography/mass spectrometry for the analysis of neutral glycosphingolipids. Biochim Biophys Acta (2011) 0.75
Initial Trabeculectomy With Mitomycin-C for Secondary Glaucoma-associated With Uveitis in Behçet Disease Patients. J Glaucoma (2017) 0.75
Primary polydipsia, but not accumulated ceramide, causes lethal renal damage in saposin D-deficient mice. Am J Physiol Renal Physiol (2012) 0.75