Published in Hum Gene Ther on August 01, 2005
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Fusion between human mesenchymal stem cells and rodent cerebellar Purkinje cells. Neuropathol Appl Neurobiol (2011) 0.92
Cell fusion in the brain: two cells forward, one cell back. Acta Neuropathol (2014) 0.89
Bone-marrow-derived mesenchymal stem cells promote proliferation and neuronal differentiation of Niemann-Pick type C mouse neural stem cells by upregulation and secretion of CCL2. Hum Gene Ther (2013) 0.88
Cellular fusion for gene delivery to SCA1 affected Purkinje neurons. Mol Cell Neurosci (2011) 0.85
Systemic Injection of RPE65-Programmed Bone Marrow-Derived Cells Prevents Progression of Chronic Retinal Degeneration. Mol Ther (2017) 0.81
Pathological roles of the VEGF/SphK pathway in Niemann-Pick type C neurons. Nat Commun (2014) 0.79
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Neuropeptide Y-based recombinant peptides ameliorate bone loss in mice by regulating hematopoietic stem/progenitor cell mobilization. BMB Rep (2017) 0.75
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Stem Cell Therapy for the Central Nervous System in Lysosomal Storage Diseases. Hum Gene Ther (2016) 0.75
Cell Fusion along the Anterior-Posterior Neuroaxis in Mice with Experimental Autoimmune Encephalomyelitis. PLoS One (2015) 0.75
Fusion of nerve fibers. Dokl Biol Sci (2007) 0.75
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nat Med (2008) 3.86
Control of endothelial targeting and intracellular delivery of therapeutic enzymes by modulating the size and shape of ICAM-1-targeted carriers. Mol Ther (2008) 2.69
The sphingosine 1-phosphate receptor 1 causes tissue retention by inhibiting the entry of peripheral tissue T lymphocytes into afferent lymphatics. Nat Immunol (2007) 2.13
A bilirubin-inducible fluorescent protein from eel muscle. Cell (2013) 2.00
Deregulation of sphingolipid metabolism in Alzheimer's disease. Neurobiol Aging (2008) 1.89
Acid sphingomyelinase activity triggers microparticle release from glial cells. EMBO J (2009) 1.78
The demographics and distribution of type B Niemann-Pick disease: novel mutations lead to new genotype/phenotype correlations. Am J Hum Genet (2002) 1.74
The unexpected role of acid sphingomyelinase in cell death and the pathophysiology of common diseases. FASEB J (2008) 1.73
Enzyme replacement and enhancement therapies: lessons from lysosomal disorders. Nat Rev Genet (2002) 1.72
Mechanism of glycosaminoglycan-mediated bone and joint disease: implications for the mucopolysaccharidoses and other connective tissue diseases. Am J Pathol (2007) 1.67
Joint and bone disease in mucopolysaccharidoses VI and VII: identification of new therapeutic targets and biomarkers using animal models. Pediatr Res (2005) 1.51
Intracerebral transplantation of bone marrow-derived mesenchymal stem cells reduces amyloid-beta deposition and rescues memory deficits in Alzheimer's disease mice by modulation of immune responses. Stem Cells (2010) 1.50
A role for ceramide in driving cancer cell resistance to doxorubicin. FASEB J (2008) 1.44
Intracerebral transplantation of mesenchymal stem cells into acid sphingomyelinase-deficient mice delays the onset of neurological abnormalities and extends their life span. J Clin Invest (2002) 1.43
Extracellular signal-regulated kinase 2 (ERK2) knockdown mice show deficits in long-term memory; ERK2 has a specific function in learning and memory. J Neurosci (2007) 1.41
AAV vector-mediated correction of brain pathology in a mouse model of Niemann-Pick A disease. Mol Ther (2005) 1.41
Acid ceramidase and human disease. Biochim Biophys Acta (2006) 1.40
Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses. Proc Natl Acad Sci U S A (2009) 1.37
Characterization of common SMPD1 mutations causing types A and B Niemann-Pick disease and generation of mutation-specific mouse models. Mol Genet Metab (2008) 1.37
Deficiency of schnurri-2, an MHC enhancer binding protein, induces mild chronic inflammation in the brain and confers molecular, neuronal, and behavioral phenotypes related to schizophrenia. Neuropsychopharmacology (2013) 1.33
Metabolic remodeling induced by mitochondrial aldehyde stress stimulates tolerance to oxidative stress in the heart. Circ Res (2009) 1.31
Human umbilical cord blood-derived mesenchymal stem cells improve neuropathology and cognitive impairment in an Alzheimer's disease mouse model through modulation of neuroinflammation. Neurobiol Aging (2010) 1.31
Bone marrow-derived mesenchymal stem cells promote neuronal networks with functional synaptic transmission after transplantation into mice with neurodegeneration. Stem Cells (2007) 1.30
Lysosomal enzyme delivery by ICAM-1-targeted nanocarriers bypassing glycosylation- and clathrin-dependent endocytosis. Mol Ther (2005) 1.29
Delivery of acid sphingomyelinase in normal and niemann-pick disease mice using intercellular adhesion molecule-1-targeted polymer nanocarriers. J Pharmacol Exp Ther (2008) 1.28
Glial protein S100B modulates long-term neuronal synaptic plasticity. Proc Natl Acad Sci U S A (2002) 1.27
A lipid-specific toxin reveals heterogeneity of sphingomyelin-containing membranes. Biophys J (2004) 1.27
Combination brain and systemic injections of AAV provide maximal functional and survival benefits in the Niemann-Pick mouse. Proc Natl Acad Sci U S A (2007) 1.27
Targeted disruption of the mouse 3-phosphoglycerate dehydrogenase gene causes severe neurodevelopmental defects and results in embryonic lethality. J Biol Chem (2003) 1.26
Acid sphingomyelinase promotes lipoprotein retention within early atheromata and accelerates lesion progression. Arterioscler Thromb Vasc Biol (2008) 1.26
The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study. Pediatrics (2004) 1.25
Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase. J Biol Chem (2003) 1.24
A fluorescence-based, high-performance liquid chromatographic assay to determine acid sphingomyelinase activity and diagnose types A and B Niemann-Pick disease. Anal Biochem (2003) 1.23
Lipid abnormalities in children with types A and B Niemann Pick disease. J Pediatr (2004) 1.22
Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics (2002) 1.22
The therapeutic potential of human umbilical cord blood-derived mesenchymal stem cells in Alzheimer's disease. Neurosci Lett (2010) 1.21
Bone marrow-derived mesenchymal stem cells reduce brain amyloid-beta deposition and accelerate the activation of microglia in an acutely induced Alzheimer's disease mouse model. Neurosci Lett (2008) 1.17
ERK2 contributes to the control of social behaviors in mice. J Neurosci (2011) 1.17
Characterization of mRNA species that are associated with postsynaptic density fraction by gene chip microarray analysis. Neurosci Res (2006) 1.16
Interaction of LDL receptor-related protein 4 (LRP4) with postsynaptic scaffold proteins via its C-terminal PDZ domain-binding motif, and its regulation by Ca/calmodulin-dependent protein kinase II. Eur J Neurosci (2006) 1.15
A fluorescence-based, high-throughput sphingomyelin assay for the analysis of Niemann-Pick disease and other disorders of sphingomyelin metabolism. Anal Biochem (2002) 1.14
Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect. Traffic (2010) 1.14
Gene transfer of human acid sphingomyelinase corrects neuropathology and motor deficits in a mouse model of Niemann-Pick type A disease. Proc Natl Acad Sci U S A (2005) 1.14
Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann-Pick A disease. Exp Neurol (2008) 1.13
Acid ceramidase is a novel factor required for early embryo survival. FASEB J (2007) 1.13
L347P PINK1 mutant that fails to bind to Hsp90/Cdc37 chaperones is rapidly degraded in a proteasome-dependent manner. Neurosci Res (2008) 1.13
Imprinting at the SMPD1 locus: implications for acid sphingomyelinase-deficient Niemann-Pick disease. Am J Hum Genet (2006) 1.12
Autoproteolytic cleavage and activation of human acid ceramidase. J Biol Chem (2008) 1.10
Oxidative stress is responsible for deficient survival and dendritogenesis in purkinje neurons from ataxia-telangiectasia mutated mutant mice. J Neurosci (2003) 1.08
Inducible cAMP early repressor (ICER) and brain functions. Mol Neurobiol (2009) 1.08
Glucosylceramide synthesis and synthase expression protect against ceramide-induced stress. J Lipid Res (2002) 1.07
A Drosophila orphan G protein-coupled receptor BOSS functions as a glucose-responding receptor: loss of boss causes abnormal energy metabolism. Proc Natl Acad Sci U S A (2008) 1.06
The reverse activity of human acid ceramidase. J Biol Chem (2003) 1.06
Simultaneous quantitative analysis of ceramide and sphingosine in mouse blood by naphthalene-2,3-dicarboxyaldehyde derivatization after hydrolysis with ceramidase. Anal Biochem (2005) 1.04
Drosophila glucosylceramide synthase: a negative regulator of cell death mediated by proapoptotic factors. J Biol Chem (2004) 1.04
Identification and characterization of human ethanolaminephosphotransferase1. J Lipid Res (2006) 1.01
Anti-TNF-alpha therapy enhances the effects of enzyme replacement therapy in rats with mucopolysaccharidosis type VI. PLoS One (2011) 1.01
Alveolar lipoproteinosis in an acid sphingomyelinase-deficient mouse model of Niemann-Pick disease. Am J Physiol Lung Cell Mol Physiol (2002) 1.01
Brain-specific Phgdh deletion reveals a pivotal role for L-serine biosynthesis in controlling the level of D-serine, an N-methyl-D-aspartate receptor co-agonist, in adult brain. J Biol Chem (2010) 1.01
Morbidity and mortality in type B Niemann-Pick disease. Genet Med (2013) 1.00
Reproductive pathology and sperm physiology in acid sphingomyelinase-deficient mice. Am J Pathol (2002) 1.00
Roles of l-serine and sphingolipid synthesis in brain development and neuronal survival. Prog Lipid Res (2008) 0.99
Quantitative analysis of sphingosine-1-phosphate by HPLC after napthalene-2,3-dicarboxaldehyde (NDA) derivatization. J Chromatogr B Analyt Technol Biomed Life Sci (2009) 0.99
Canopy1, a positive feedback regulator of FGF signaling, controls progenitor cell clustering during Kupffer's vesicle organogenesis. Proc Natl Acad Sci U S A (2011) 0.99
Expression of Btcl2, a novel member of Btcl gene family, during development of the central nervous system. Brain Res Dev Brain Res (2004) 0.98
Identification and characterization of eight novel SMPD1 mutations causing types A and B Niemann-Pick disease. Mol Med (2010) 0.98
Soluble CCL5 derived from bone marrow-derived mesenchymal stem cells and activated by amyloid β ameliorates Alzheimer's disease in mice by recruiting bone marrow-induced microglia immune responses. Stem Cells (2012) 0.98
The microtubule destabilizer stathmin mediates the development of dendritic arbors in neuronal cells. J Cell Sci (2007) 0.98
Nitric oxide boosts chemoimmunotherapy via inhibition of acid sphingomyelinase in a mouse model of melanoma. Cancer Res (2007) 0.98
An endogenous serine/threonine protein phosphatase inhibitor, G-substrate, reduces vulnerability in models of Parkinson's disease. J Neurosci (2007) 0.98
Inducible cAMP early repressor acts as a negative regulator for kindling epileptogenesis and long-term fear memory. J Neurosci (2008) 0.98
A cellular deficiency of gangliosides causes hypersensitivity to Clostridium perfringens phospholipase C. J Biol Chem (2005) 0.98
A novel gene, Btcl1, encoding CUB and LDLa domains is expressed in restricted areas of mouse brain. Biochem Biophys Res Commun (2003) 0.97
AAV8-mediated hepatic expression of acid sphingomyelinase corrects the metabolic defect in the visceral organs of a mouse model of Niemann-Pick disease. Mol Ther (2005) 0.97
Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice. J Neurochem (2011) 0.97
Ocular manifestations of Niemann-Pick disease type B. Ophthalmology (2004) 0.96
Identification and characterization of SMPD1 mutations causing Niemann-Pick types A and B in Spanish patients. Hum Mutat (2009) 0.95
D-serine in glia and neurons derives from 3-phosphoglycerate dehydrogenase. J Neurosci (2013) 0.95
Assembly of hereditary amyloid beta-protein variants in the presence of favorable gangliosides. FEBS Lett (2005) 0.95
Mannose 6-phosphate receptor-mediated uptake is defective in acid sphingomyelinase-deficient macrophages: implications for Niemann-Pick disease enzyme replacement therapy. J Biol Chem (2003) 0.95
Bone marrow transplantation for feline mucopolysaccharidosis I. Mol Genet Metab (2007) 0.95
Systemic ceramide accumulation leads to severe and varied pathological consequences. EMBO Mol Med (2013) 0.94
Comparative binding, endocytosis, and biodistribution of antibodies and antibody-coated carriers for targeted delivery of lysosomal enzymes to ICAM-1 versus transferrin receptor. J Inherit Metab Dis (2012) 0.93
Neuropathology of the acid sphingomyelinase knockout mouse model of Niemann-Pick A disease including structure-function studies associated with cerebellar Purkinje cell degeneration. Exp Neurol (2008) 0.93
Ceramide reduction and transcriptional up-regulation of glucosylceramide synthase through doxorubicin-activated Sp1 in drug-resistant HL-60/ADR cells. Cancer Res (2004) 0.93
Ceramide glucosyltransferase of the nematode Caenorhabditis elegans is involved in oocyte formation and in early embryonic cell division. Glycobiology (2011) 0.93
ERK1 plays a critical protective role against N-methyl-D-aspartate-induced retinal injury. J Neurosci Res (2008) 0.92
New insights on glucosylated lipids: metabolism and functions. Biochim Biophys Acta (2013) 0.92
Acid sphingomyelinase overexpression enhances the antineoplastic effects of irradiation in vitro and in vivo. Mol Ther (2008) 0.91
Construction of conditional acid ceramidase knockout mice and in vivo effects on oocyte development and fertility. Cell Physiol Biochem (2012) 0.90
Bone marrow-derived mesenchymal stem cells prevent the loss of Niemann-Pick type C mouse Purkinje neurons by correcting sphingolipid metabolism and increasing sphingosine-1-phosphate. Stem Cells (2010) 0.90