Published in J Neurochem on April 17, 2007
Mass spectrometric and computational analysis of cytokine-induced alterations in the astrocyte secretome. Proteomics (2009) 1.17
AAV-mediated gene delivery in adult GM1-gangliosidosis mice corrects lysosomal storage in CNS and improves survival. PLoS One (2010) 0.96
Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease. Behav Brain Res (2008) 0.90
Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff disease. Lipids (2008) 0.88
Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease. Behav Brain Res (2015) 0.78
Apprehending ganglioside diversity: a comprehensive methodological approach. J Lipid Res (2015) 0.76
Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis. ASN Neuro (2015) 0.75
An Experimental Animal Model of Photodynamic Optic Nerve Head Injury (PONHI). Curr Eye Res (2016) 0.75
Ganglioside Profiling of the Human Retina: Comparison with Other Ocular Structures, Brain and Plasma Reveals Tissue Specificities. PLoS One (2016) 0.75
Fission and selective fusion govern mitochondrial segregation and elimination by autophagy. EMBO J (2008) 12.89
Cardiotoxicity of the cancer therapeutic agent imatinib mesylate. Nat Med (2006) 9.07
Imagable 4T1 model for the study of late stage breast cancer. BMC Cancer (2008) 3.03
Cyclin D1-dependent kinase activity in murine development and mammary tumorigenesis. Cancer Cell (2006) 2.91
Clinical trial of lutein in patients with retinitis pigmentosa receiving vitamin A. Arch Ophthalmol (2010) 2.78
Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. Nat Med (2007) 2.57
Defects in RGS9 or its anchor protein R9AP in patients with slow photoreceptor deactivation. Nature (2004) 2.48
Night blindness and abnormal cone electroretinogram ON responses in patients with mutations in the GRM6 gene encoding mGluR6. Proc Natl Acad Sci U S A (2005) 2.36
ω-3 intake and visual acuity in patients with retinitis pigmentosa receiving vitamin A. Arch Ophthalmol (2012) 2.23
RP2 and RPGR mutations and clinical correlations in patients with X-linked retinitis pigmentosa. Am J Hum Genet (2003) 1.83
Further evaluation of docosahexaenoic acid in patients with retinitis pigmentosa receiving vitamin A treatment: subgroup analyses. Arch Ophthalmol (2004) 1.80
The calorically restricted ketogenic diet, an effective alternative therapy for malignant brain cancer. Nutr Metab (Lond) (2007) 1.75
Shared mutations in NR2E3 in enhanced S-cone syndrome, Goldmann-Favre syndrome, and many cases of clumped pigmentary retinal degeneration. Arch Ophthalmol (2003) 1.74
Clinical trial of docosahexaenoic acid in patients with retinitis pigmentosa receiving vitamin A treatment. Arch Ophthalmol (2004) 1.71
GM1-ganglioside accumulation at the mitochondria-associated ER membranes links ER stress to Ca(2+)-dependent mitochondrial apoptosis. Mol Cell (2009) 1.66
GM1-ganglioside-mediated activation of the unfolded protein response causes neuronal death in a neurodegenerative gangliosidosis. Mol Cell (2004) 1.64
Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor. EMBO J (2003) 1.60
AIPL1, the protein that is defective in Leber congenital amaurosis, is essential for the biosynthesis of retinal rod cGMP phosphodiesterase. Proc Natl Acad Sci U S A (2004) 1.54
Cone cGMP-gated channel mutations and clinical findings in patients with achromatopsia, macular degeneration, and other hereditary cone diseases. Hum Mutat (2005) 1.52
Lack of scientific rationale for use of valproic acid for retinitis pigmentosa. Br J Ophthalmol (2010) 1.52
Disease progression in patients with dominant retinitis pigmentosa and rhodopsin mutations. Invest Ophthalmol Vis Sci (2002) 1.49
Gene replacement therapy rescues photoreceptor degeneration in a murine model of Leber congenital amaurosis lacking RPGRIP. Invest Ophthalmol Vis Sci (2005) 1.47
Metabolic management of glioblastoma multiforme using standard therapy together with a restricted ketogenic diet: Case Report. Nutr Metab (Lond) (2010) 1.46
Cardiolipin and electron transport chain abnormalities in mouse brain tumor mitochondria: lipidomic evidence supporting the Warburg theory of cancer. J Lipid Res (2008) 1.44
AAV-mediated expression targeting of rod and cone photoreceptors with a human rhodopsin kinase promoter. Invest Ophthalmol Vis Sci (2007) 1.44
Recessive NRL mutations in patients with clumped pigmentary retinal degeneration and relative preservation of blue cone function. Proc Natl Acad Sci U S A (2004) 1.42
Antiangiogenic and proapoptotic effects of dietary restriction on experimental mouse and human brain tumors. Clin Cancer Res (2004) 1.37
Perspectives on the metabolic management of epilepsy through dietary reduction of glucose and elevation of ketone bodies. J Neurochem (2003) 1.36
Collagen XVIII/endostatin is essential for vision and retinal pigment epithelial function. EMBO J (2003) 1.34
Pericyte deficiencies lead to aberrant tumor vascularizaton in the brain of the NG2 null mouse. Dev Biol (2010) 1.34
Lipidomic analysis and electron transport chain activities in C57BL/6J mouse brain mitochondria. J Neurochem (2008) 1.29
Expression specificity of GFAP transgenes. Neurochem Res (2004) 1.29
Neuraminidase 1 is a negative regulator of lysosomal exocytosis. Dev Cell (2008) 1.24
Management of multifactorial idiopathic epilepsy in EL mice with caloric restriction and the ketogenic diet: role of glucose and ketone bodies. Nutr Metab (Lond) (2004) 1.22
Up-regulation of NG2 proteoglycan and interferon-induced transmembrane proteins 1 and 3 in mouse astrocytoma: a membrane proteomics approach. Cancer Lett (2008) 1.22
Replacement gene therapy with a human RPGRIP1 sequence slows photoreceptor degeneration in a murine model of Leber congenital amaurosis. Hum Gene Ther (2010) 1.19
A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice. Mol Ther (2013) 1.19
Identification of immunologic and pathologic parameters of death versus survival in respiratory tularemia. Infect Immun (2007) 1.15
Mechanisms of murine lacrimal gland repair after experimentally induced inflammation. Invest Ophthalmol Vis Sci (2008) 1.14
Screen of the IMPDH1 gene among patients with dominant retinitis pigmentosa and clinical features associated with the most common mutation, Asp226Asn. Invest Ophthalmol Vis Sci (2005) 1.13
Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice. Hum Mol Genet (2002) 1.13
Metastatic cancer cells with macrophage properties: evidence from a new murine tumor model. Int J Cancer (2008) 1.13
Caloric restriction in C57BL/6J mice mimics therapeutic fasting in humans. Lipids Health Dis (2006) 1.12
Differentiating N-linked glycan structural isomers in metastatic and nonmetastatic tumor cells using sequential mass spectrometry. Glycobiology (2008) 1.11
A single, abbreviated RPGR-ORF15 variant reconstitutes RPGR function in vivo. Invest Ophthalmol Vis Sci (2005) 1.11
Optical coherence tomography findings in occult macular dystrophy. Am J Ophthalmol (2006) 1.08
Drug/diet synergy for managing malignant astrocytoma in mice: 2-deoxy-D-glucose and the restricted ketogenic diet. Nutr Metab (Lond) (2008) 1.07
Dominant, gain-of-function mutant produced by truncation of RPGR. Invest Ophthalmol Vis Sci (2004) 1.07
Itaconic acid is a mammalian metabolite induced during macrophage activation. J Am Chem Soc (2011) 1.06
Absence of photoreceptor rescue with D-cis-diltiazem in the rd mouse. Invest Ophthalmol Vis Sci (2002) 1.06
Functional amelioration of murine galactosialidosis by genetically modified bone marrow hematopoietic progenitor cells. Blood (2002) 1.05
Dynamic simulation of cardiolipin remodeling: greasing the wheels for an interpretative approach to lipidomics. J Lipid Res (2010) 1.05
Differential effects of energy stress on AMPK phosphorylation and apoptosis in experimental brain tumor and normal brain. Mol Cancer (2008) 1.04
Akt-dependent proapoptotic effects of dietary restriction on late-stage management of a phosphatase and tensin homologue/tuberous sclerosis complex 2-deficient mouse astrocytoma. Clin Cancer Res (2008) 1.04
LPS-induced cytokine production in human dendritic cells is regulated by sialidase activity. J Leukoc Biol (2010) 1.03
A novel pre-clinical in vivo mouse model for malignant brain tumor growth and invasion. J Neurooncol (2010) 1.03
Chemokine-induced recruitment of genetically modified bone marrow cells into the CNS of GM1-gangliosidosis mice corrects neuronal pathology. Blood (2005) 1.03
The ketogenic diet and hyperbaric oxygen therapy prolong survival in mice with systemic metastatic cancer. PLoS One (2013) 1.02
Role of beta-galactosidase and elastin binding protein in lysosomal and nonlysosomal complexes of patients with GM1-gangliosidosis. Hum Mutat (2005) 1.01
Hypothesis: are neoplastic macrophages/microglia present in glioblastoma multiforme? ASN Neuro (2011) 1.00
Encephalopathy caused by ablation of very long acyl chain ceramide synthesis may be largely due to reduced galactosylceramide levels. J Biol Chem (2011) 0.99
Lysosomal multienzyme complex: pros and cons of working together. Cell Mol Life Sci (2013) 0.99
Primary intrapulmonary spindle cell thymoma with marked granulomatous reaction: report of a case with review of literature. Int J Surg Pathol (2003) 0.98
Therapeutic response in feline sandhoff disease despite immunity to intracranial gene therapy. Mol Ther (2013) 0.97
GM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings. Biochim Biophys Acta (2011) 0.97
Beta2 integrins control the severity of murine Lyme carditis. Infect Immun (2005) 0.97
Novel mutations in the NRL gene and associated clinical findings in patients with dominant retinitis pigmentosa. Arch Ophthalmol (2002) 0.96
Natural course of ocular function in pigmented paravenous retinochoroidal atrophy. Am J Ophthalmol (2006) 0.96
AAV-mediated gene delivery in adult GM1-gangliosidosis mice corrects lysosomal storage in CNS and improves survival. PLoS One (2010) 0.96
Glutamine targeting inhibits systemic metastasis in the VM-M3 murine tumor model. Int J Cancer (2010) 0.95
Effects of low AIPL1 expression on phototransduction in rods. Invest Ophthalmol Vis Sci (2006) 0.95
Glucose-dependent de novo lipogenesis in B lymphocytes: a requirement for atp-citrate lyase in lipopolysaccharide-induced differentiation. J Biol Chem (2014) 0.95
N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis. J Neurochem (2004) 0.94
Enhanced virulence of Chlamydia muridarum respiratory infections in the absence of TLR2 activation. PLoS One (2011) 0.94
Sexual dysfunction and sudden death in epileptic male EL mice: inheritance and prevention with the ketogenic diet. Epilepsia (2003) 0.93
Glycolipid and ganglioside metabolism imbalances in Huntington's disease. Neurobiol Dis (2007) 0.93
Genotype-phenotype correlations in Bardet-Biedl syndrome. Arch Ophthalmol (2012) 0.93
Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice. J Neurosci Res (2006) 0.93
Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis. Nat Commun (2013) 0.92
Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA/2J and knockout mice. Biochem Genet (2004) 0.92
Thematic review series: sphingolipids. Ganglioside GM3 suppresses the proangiogenic effects of vascular endothelial growth factor and ganglioside GD1a. J Lipid Res (2008) 0.92
Novel mutations in the KCNV2 gene in patients with cone dystrophy and a supernormal rod electroretinogram. Ophthalmic Genet (2007) 0.92
N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice. Neurochem Int (2007) 0.92
Aggravated Lyme carditis in CD11a-/- and CD11c-/- mice. Infect Immun (2005) 0.92
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis. Mol Genet Metab (2008) 0.92
Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue. Biochim Biophys Acta (2010) 0.92
Oxygenation prevents sudden death in seizure-prone mice. Epilepsia (2004) 0.91
Rod and cone opsin mislocalization in an autopsy eye from a carrier of X-linked retinitis pigmentosa with a Gly436Asp mutation in the RPGR gene. Am J Ophthalmol (2006) 0.91
New mutations in the PPBG gene lead to loss of PPCA protein which affects the level of the beta-galactosidase/neuraminidase complex and the EBP-receptor. Mol Genet Metab (2004) 0.91
Influence of caloric restriction on constitutive expression of NF-κB in an experimental mouse astrocytoma. PLoS One (2011) 0.91
Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain. J Lipid Res (2011) 0.90
Hippocampal neurons and glia in epileptic EL mice. J Neurocytol (2003) 0.90
Calorie restriction as an anti-invasive therapy for malignant brain cancer in the VM mouse. ASN Neuro (2010) 0.90
Galactosialidosis: review and analysis of CTSA gene mutations. Orphanet J Rare Dis (2013) 0.90
The potential action of galactose as a "chemical chaperone": increase of beta galactosidase activity in fibroblasts from an adult GM1-gangliosidosis patient. Eur J Paediatr Neurol (2008) 0.90
S100 protein expression distinguishes metanephric adenomas from other renal neoplasms. Pathol Res Pract (2008) 0.89