Peripheral nervous system manifestations in a Sandhoff disease mouse model: nerve conduction, myelin structure, lipid analysis.

PubWeight™: 0.79‹?›

🔗 View Article (PMC 1976615)

Published in J Negat Results Biomed on July 10, 2007

Authors

Melanie A McNally1, Rena C Baek, Robin L Avila, Thomas N Seyfried, Gary R Strichartz, Daniel A Kirschner

Author Affiliations

1: Biology Department, Boston College, 140 Commonwealth Avenue, Chestnut Hill, MA 02467, USA. melanie.mcnally@gmail.com

Articles cited by this

A simple method for the isolation and purification of total lipides from animal tissues. J Biol Chem (1957) 173.99

Prevalence of lysosomal storage disorders. JAMA (1999) 9.06

Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nat Genet (1995) 2.64

Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation. Proc Natl Acad Sci U S A (2000) 2.11

Analysis of brain lipids by high performance thin-layer chromatography and densitometry. J Lipid Res (1983) 1.71

Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases. Hum Mol Genet (1997) 1.56

Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. J Clin Invest (1998) 1.49

Different intracellular pathomechanisms produce diverse Myelin Protein Zero neuropathies in transgenic mice. J Neurosci (2006) 1.34

Evolution of a neuroprotective function of central nervous system myelin. J Cell Biol (2006) 1.25

Designation and schematic structure of gangliosides and allied glycosphingolipids. Prog Brain Res (1994) 1.21

Structure and stability of internodal myelin in mouse models of hereditary neuropathy. J Neuropathol Exp Neurol (2005) 1.16

GM-2 gangliosidosis (Sandhoff's disease): two year follow-up by MRI. Neuroradiology (1994) 1.14

Membrane structure in isolated and intact myelins. Biophys J (1989) 1.11

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis. Neuropathol Appl Neurobiol (2002) 1.11

Dysmyelinogenesis in animal model of GM1 gangliosidosis. Pediatr Neurol (1992) 1.06

White matter changes associated with feline GM2 gangliosidosis (Sandhoff disease): correlation of MR findings with pathologic and ultrastructural abnormalities. AJNR Am J Neuroradiol (1995) 1.06

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease. Neurobiol Dis (2004) 1.04

Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice. J Lipid Res (2005) 1.02

Cerebral, cerebellar, and brain stem gangliosides in mice susceptible to audiogenic seizures. J Neurochem (1978) 1.02

New therapeutic prospects for the glycosphingolipid lysosomal storage diseases. Biochem Pharmacol (1998) 1.00

Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice. J Neurosci Res (2006) 0.93

N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice. Neurochem Int (2007) 0.92

Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA/2J and knockout mice. Biochem Genet (2004) 0.92

Composition and metabolism of gangliosides in rat peripheral nervous system during development. J Neurochem (1982) 0.89

The clinical aspects of adult hexosaminidase deficiencies. Dev Neurosci (1991) 0.88

Differential cellular enrichment of gangliosides in the mouse cerebellum: analysis using neurological mutants. J Neurochem (1982) 0.86

Lysosomal storage results in impaired survival but normal neurite outgrowth in dorsal root ganglion neurones from a mouse model of Sandhoff disease. Neuropathol Appl Neurobiol (2002) 0.83

Neurophysiological study in chronic GM2 gangliosidosis (hexosaminidase A and B deficiency), with motor neuron disease phenotype. Ital J Neurol Sci (1989) 0.82

Establishment of immortalized Schwann cells from Sandhoff mice and corrective effect of recombinant human beta-hexosaminidase A on the accumulated GM2 ganglioside. J Hum Genet (2005) 0.79

Peripheral and autonomic nervous system involvement in chronic GM2-gangliosidosis. J Inherit Metab Dis (2001) 0.79

Targeting the hexosaminidase genes: mouse models of the GM2 gangliosidoses. Adv Genet (2001) 0.77

Articles by these authors

MAP kinase and pain. Brain Res Rev (2008) 3.75

Constitutively active Akt induces enhanced myelination in the CNS. J Neurosci (2008) 2.60

A peptide c-Jun N-terminal kinase (JNK) inhibitor blocks mechanical allodynia after spinal nerve ligation: respective roles of JNK activation in primary sensory neurons and spinal astrocytes for neuropathic pain development and maintenance. J Neurosci (2006) 2.59

Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease. Nat Med (2007) 2.57

Reduction of postincisional allodynia by subcutaneous bupivacaine: findings with a new model in the hairy skin of the rat. Anesthesiology (2005) 1.80

The calorically restricted ketogenic diet, an effective alternative therapy for malignant brain cancer. Nutr Metab (Lond) (2007) 1.75

The role of sodium channels in chronic inflammatory and neuropathic pain. J Pain (2006) 1.59

Endothelin-B receptor activation triggers an endogenous analgesic cascade at sites of peripheral injury. Nat Med (2003) 1.54

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

The formation of straight and twisted filaments from short tau peptides. J Biol Chem (2004) 1.43

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

Different intracellular pathomechanisms produce diverse Myelin Protein Zero neuropathies in transgenic mice. J Neurosci (2006) 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

Evolution of a neuroprotective function of central nervous system myelin. J Cell Biol (2006) 1.25

A genetic mouse model of adult-onset, pervasive central nervous system demyelination with robust remyelination. Brain (2010) 1.24

Polyglutamine homopolymers having 8-45 residues form slablike beta-crystallite assemblies. Proteins (2005) 1.22

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

A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice. Mol Ther (2013) 1.19

Structure and stability of internodal myelin in mouse models of hereditary neuropathy. J Neuropathol Exp Neurol (2005) 1.16

Tetraspanin CD9 is a "proteolipid," and its interaction with alpha 3 integrin in microdomain is promoted by GM3 ganglioside, leading to inhibition of laminin-5-dependent cell motility. J Biol Chem (2002) 1.15

Formation of amyloid fibrils in vitro by human gammaD-crystallin and its isolated domains. Mol Vis (2008) 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

Filaments of the Ure2p prion protein have a cross-beta core structure. J Struct Biol (2005) 1.09

Structure of core domain of fibril-forming PHF/Tau fragments. Biophys J (2005) 1.09

Drug/diet synergy for managing malignant astrocytoma in mice: 2-deoxy-D-glucose and the restricted ketogenic diet. Nutr Metab (Lond) (2008) 1.07

Structural properties of Gerstmann-Straussler-Scheinker disease amyloid protein. J Biol Chem (2003) 1.06

Itaconic acid is a mammalian metabolite induced during macrophage activation. J Am Chem Soc (2011) 1.06

Cotinine reduces amyloid-β aggregation and improves memory in Alzheimer's disease mice. J Alzheimers Dis (2011) 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

Local injection of a selective endothelin-B receptor agonist inhibits endothelin-1-induced pain-like behavior and excitation of nociceptors in a naloxone-sensitive manner. J Neurosci (2002) 1.03

The critical role of concentration for lidocaine block of peripheral nerve in vivo: studies of function and drug uptake in the rat. Anesthesiology (2003) 1.03

A novel pre-clinical in vivo mouse model for malignant brain tumor growth and invasion. J Neurooncol (2010) 1.03

Internodal myelination during development quantitated using X-ray diffraction. J Struct Biol (2009) 1.02

The ketogenic diet and hyperbaric oxygen therapy prolong survival in mice with systemic metastatic cancer. PLoS One (2013) 1.02

ZFP191 is required by oligodendrocytes for CNS myelination. Genes Dev (2010) 1.01

Tetracaine-membrane interactions: effects of lipid composition and phase on drug partitioning, location, and ionization. Biophys J (2007) 1.00

Hypothesis: are neoplastic macrophages/microglia present in glioblastoma multiforme? ASN Neuro (2011) 1.00

On the mechanism by which epinephrine potentiates lidocaine's peripheral nerve block. Anesthesiology (2003) 0.99

Therapeutic response in feline sandhoff disease despite immunity to intracranial gene therapy. Mol Ther (2013) 0.97

Tactile allodynia initiated by local subcutaneous endothelin-1 is prolonged by activation of TRPV-1 receptors. Exp Biol Med (Maywood) (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

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

Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice. J Neurochem (2007) 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

Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice. J Neurosci Res (2006) 0.93

Differential effects of Phe19 and Phe20 on fibril formation by amyloidogenic peptide A beta 16-22 (Ac-KLVFFAE-NH2). Proteins (2010) 0.92

Poly-(L-alanine) expansions form core beta-sheets that nucleate amyloid assembly. Proteins (2005) 0.92

Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA/2J and knockout mice. Biochem Genet (2004) 0.92

N-butyldeoxygalactonojirimycin reduces brain ganglioside and GM2 content in neonatal Sandhoff disease mice. Neurochem Int (2007) 0.92

Multiple phases of relief from experimental mechanical allodynia by systemic lidocaine: responses to early and late infusions. Pain (2003) 0.92

Alzheimer's beta-amyloid: insights into fibril formation and structure from Congo red binding. Subcell Biochem (2005) 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

Air-stimulated ATP release from keratinocytes occurs through connexin hemichannels. PLoS One (2013) 0.91

Peripheral nervous system plasmalogens regulate Schwann cell differentiation and myelination. J Clin Invest (2014) 0.91

Influence of caloric restriction on constitutive expression of NF-κB in an experimental mouse astrocytoma. PLoS One (2011) 0.91

Oxygenation prevents sudden death in seizure-prone mice. Epilepsia (2004) 0.91

Lack of collagen XV impairs peripheral nerve maturation and, when combined with laminin-411 deficiency, leads to basement membrane abnormalities and sensorimotor dysfunction. J Neurosci (2010) 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

Sensory fibers resistant to the actions of tetrodotoxin mediate nocifensive responses to local administration of endothelin-1 in rats. Pain (2004) 0.89

Influence of methotrexate and cisplatin on tumor progression and survival in the VM mouse model of systemic metastatic cancer. Int J Cancer (2010) 0.89

Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff disease. Lipids (2008) 0.88

Myelin structure and composition in zebrafish. Neurochem Res (2006) 0.87

Cutaneous endothelin-A receptors elevate post-incisional pain. Pain (2007) 0.87

Examination of the brain mitochondrial lipidome using shotgun lipidomics. Methods Mol Biol (2009) 0.87

Myelin protein zero exists as dimers and tetramers in native membranes of Xenopus laevis peripheral nerve. J Neurosci Res (2002) 0.87

Micromolar lidocaine selectively blocks propagating ectopic impulses at a distance from their site of origin. Pain (2002) 0.86

Improvement in motor and exploratory behavior in Rett syndrome mice with restricted ketogenic and standard diets. Epilepsy Behav (2009) 0.86

Cytoplasmic domain of human myelin protein zero likely folded as beta-structure in compact myelin. Biophys J (2006) 0.86

In vitro growth environment produces lipidomic and electron transport chain abnormalities in mitochondria from non-tumorigenic astrocytes and brain tumours. ASN Neuro (2009) 0.86

Perspectives on the mesenchymal origin of metastatic cancer. Cancer Metastasis Rev (2010) 0.86

Brain mitochondrial lipid abnormalities in mice susceptible to spontaneous gliomas. Lipids (2008) 0.86

Bupivacaine inhibits activation of neuronal spinal extracellular receptor-activated kinase through selective effects on ionotropic receptors. Anesthesiology (2006) 0.85

Absence of pathogenic mitochondrial DNA mutations in mouse brain tumors. BMC Cancer (2005) 0.85

Gene-linked shift in ganglioside distribution influences growth and vascularity in a mouse astrocytoma. J Neurochem (2006) 0.85

Gerstmann-Sträussler-Scheinker disease amyloid protein polymerizes according to the "dock-and-lock" model. J Biol Chem (2005) 0.85

Assemblies of Alzheimer's peptides A beta 25-35 and A beta 31-35: reverse-turn conformation and side-chain interactions revealed by X-ray diffraction. J Struct Biol (2003) 0.85