Encephalopathy caused by ablation of very long acyl chain ceramide synthesis may be largely due to reduced galactosylceramide levels.

PubWeight™: 0.99‹?› | Rank: Top 15%

🔗 View Article (PMC 3191043)

Published in J Biol Chem on June 24, 2011

Authors

Oshrit Ben-David1, Yael Pewzner-Jung, Ori Brenner, Elad L Laviad, Aviram Kogot-Levin, Itai Weissberg, Inbal E Biton, Reut Pienik, Elaine Wang, Samuel Kelly, Joseph Alroy, Annick Raas-Rothschild, Alon Friedman, Britta Brügger, Alfred H Merrill, Anthony H Futerman

Author Affiliations

1: Department of Biological Chemistry, Weizmann Institute of Science, Rehovot, Israel.

Articles citing this

Reduced ceramide synthase 2 activity causes progressive myoclonic epilepsy. Ann Clin Transl Neurol (2014) 1.11

Ablation of ceramide synthase 2 causes chronic oxidative stress due to disruption of the mitochondrial respiratory chain. J Biol Chem (2013) 1.11

Modulation of ceramide synthase activity via dimerization. J Biol Chem (2012) 1.02

Ablation of ceramide synthase 2 strongly affects biophysical properties of membranes. J Lipid Res (2012) 0.97

Ceramide synthases expression and role of ceramide synthase-2 in the lung: insight from human lung cells and mouse models. PLoS One (2013) 0.97

Direct tandem mass spectrometric profiling of sulfatides in dry urinary samples for screening of metachromatic leukodystrophy. Clin Chim Acta (2013) 0.87

A fluorescent assay for ceramide synthase activity. J Lipid Res (2012) 0.87

Brain lipidomes of subcortical ischemic vascular dementia and mixed dementia. Neurobiol Aging (2014) 0.85

Mutation for nonsyndromic mental retardation in the trans-2-enoyl-CoA reductase TER gene involved in fatty acid elongation impairs the enzyme activity and stability, leading to change in sphingolipid profile. J Biol Chem (2013) 0.83

Human genetic disorders of sphingolipid biosynthesis. J Inherit Metab Dis (2014) 0.81

Cell-type-specific expression pattern of ceramide synthase 2 protein in mouse tissues. Histochem Cell Biol (2013) 0.79

Sustained activation of sphingomyelin synthase by 2-hydroxyoleic acid induces sphingolipidosis in tumor cells. J Lipid Res (2013) 0.78

Chromosomal microarray analysis in clinical evaluation of neurodevelopmental disorders-reporting a novel deletion of SETDB1 and illustration of counseling challenge. Pediatr Res (2016) 0.75

The 3-hydroxyacyl-CoA dehydratases HACD1 and HACD2 exhibit functional redundancy and are active in a wide range of fatty acid elongation pathways. J Biol Chem (2017) 0.75

Articles cited by this

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

Quantitative estimation of sialic acids. II. A colorimetric resorcinol-hydrochloric acid method. Biochim Biophys Acta (1957) 11.28

Biology of oligodendrocyte and myelin in the mammalian central nervous system. Physiol Rev (2001) 5.50

Axon degeneration mechanisms: commonality amid diversity. Nat Rev Neurosci (2005) 4.70

The HIV lipidome: a raft with an unusual composition. Proc Natl Acad Sci U S A (2006) 4.15

Myelination in rat brain: method of myelin isolation. J Neurochem (1973) 4.01

When do Lasses (longevity assurance genes) become CerS (ceramide synthases)?: Insights into the regulation of ceramide synthesis. J Biol Chem (2006) 3.09

Clinical and genetic delineation of neurodegeneration with brain iron accumulation. J Med Genet (2008) 3.08

Sphingomyelin synthesis in rat liver occurs predominantly at the cis and medial cisternae of the Golgi apparatus. J Biol Chem (1990) 2.72

Quantitative analysis of sphingolipids for lipidomics using triple quadrupole and quadrupole linear ion trap mass spectrometers. J Lipid Res (2008) 2.62

Mammalian Lass6 and its related family members regulate synthesis of specific ceramides. Biochem J (2005) 2.62

Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability. Cell (1996) 2.53

Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate. J Biol Chem (2007) 2.50

Evidence for segregation of sphingomyelin and cholesterol during formation of COPI-coated vesicles. J Cell Biol (2000) 2.13

Two mammalian longevity assurance gene (LAG1) family members, trh1 and trh4, regulate dihydroceramide synthesis using different fatty acyl-CoA donors. J Biol Chem (2003) 2.06

Upstream of growth and differentiation factor 1 (uog1), a mammalian homolog of the yeast longevity assurance gene 1 (LAG1), regulates N-stearoyl-sphinganine (C18-(dihydro)ceramide) synthesis in a fumonisin B1-independent manner in mammalian cells. J Biol Chem (2002) 2.03

Mammalian ceramide synthases. IUBMB Life (2010) 1.94

Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase. Proc Natl Acad Sci U S A (1999) 1.79

Lipids of nervous tissue: composition and metabolism. Prog Lipid Res (1985) 1.66

Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. J Biol Chem (2009) 1.58

Astrocytic dysfunction in epileptogenesis: consequence of altered potassium and glutamate homeostasis? J Neurosci (2009) 1.57

A critical role for ceramide synthase 2 in liver homeostasis: I. alterations in lipid metabolic pathways. J Biol Chem (2010) 1.56

LASS3 (longevity assurance homologue 3) is a mainly testis-specific (dihydro)ceramide synthase with relatively broad substrate specificity. Biochem J (2006) 1.54

Sulfatide is essential for the maintenance of CNS myelin and axon structure. Glia (2006) 1.49

Fatty acid 2-Hydroxylation in mammalian sphingolipid biology. Biochim Biophys Acta (2009) 1.45

Huntington's Disease. Semin Neurol (2007) 1.43

The long-chain sphingoid base of sphingolipids is acylated at the cytosolic surface of the endoplasmic reticulum in rat liver. Biochem J (1993) 1.43

A critical role for ceramide synthase 2 in liver homeostasis: II. insights into molecular changes leading to hepatopathy. J Biol Chem (2010) 1.41

Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia. Exp Neurol (2003) 1.38

Kinetic characterization of mammalian ceramide synthases: determination of K(m) values towards sphinganine. FEBS Lett (2007) 1.29

The role of the ceramide acyl chain length in neurodegeneration: involvement of ceramide synthases. Neuromolecular Med (2010) 1.25

Ceramide signaling downstream of the p75 neurotrophin receptor mediates the effects of nerve growth factor on outgrowth of cultured hippocampal neurons. J Neurosci (1999) 1.24

Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration. J Neurosci (2008) 1.24

Isolation of glycosphingolipids. Methods Enzymol (1994) 1.23

Azoxymethane-induced fulminant hepatic failure in C57BL/6J mice: characterization of a new animal model. Am J Physiol (1999) 1.18

Mechanisms of axonal spheroid formation in central nervous system Wallerian degeneration. J Neuropathol Exp Neurol (2010) 1.08

Parameters related to lipid metabolism as markers of myelination in mouse brain. J Neurochem (2001) 1.06

Klotho-related protein is a novel cytosolic neutral beta-glycosylceramidase. J Biol Chem (2007) 1.06

Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2. Histochem Cell Biol (2007) 1.03

Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease. Hum Mol Genet (2011) 1.03

A carbohydrate-carbohydrate interaction between galactosylceramide-containing liposomes and cerebroside sulfate-containing liposomes: dependence on the glycolipid ceramide composition. Biochemistry (1993) 1.02

Nerve growth factor-induced p75-mediated death of cultured hippocampal neurons is age-dependent and transduced through ceramide generated by neutral sphingomyelinase. J Biol Chem (2002) 1.01

Developmentally regulated ceramide synthase 6 increases mitochondrial Ca2+ loading capacity and promotes apoptosis. J Biol Chem (2010) 1.01

Functional role of mGluR1 and mGluR4 in pilocarpine-induced temporal lobe epilepsy. Neurobiol Dis (2007) 1.00

A case of lipogranulomatosis Farber: some clinical and ultrastructural aspects. Eur J Pediatr (1985) 0.97

A regulatory role for sphingolipids in neuronal growth. Inhibition of sphingolipid synthesis and degradation have opposite effects on axonal branching. J Biol Chem (1995) 0.96

Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase. J Neurochem (2005) 0.95

Synthesis of non-hydroxy-galactosylceramides and galactosyldiglycerides by hydroxy-ceramide galactosyltransferase. Biochem J (1996) 0.94

Genetic dissection of myelin galactolipid function. J Neurocytol (2000) 0.93

Sphingolipids in multiple sclerosis. Neuromolecular Med (2010) 0.93

Galactolipids in the formation and function of the myelin sheath. Microsc Res Tech (1998) 0.90

Defective calcium homeostasis in the cerebellum in a mouse model of Niemann-Pick A disease. J Neurochem (2005) 0.89

Distinct roles for ceramide and glucosylceramide at different stages of neuronal growth. J Neurosci (1997) 0.87

Sphingolipid biosynthesis is necessary for dendrite growth and survival of cerebellar Purkinje cells in culture. J Neurochem (1995) 0.86

GM1-gangliosidosis in Alaskan huskies: clinical and pathologic findings. Vet Pathol (2001) 0.86

Oligodendrocyte-specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT-deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide level. Glia (2005) 0.85

Suppressed UDP-galactose: ceramide galactosyltransferase and myelin protein mRNA in twitcher mouse brain. J Neurosci Res (1998) 0.81

Elevated sulfatide levels in neurons cause lethal audiogenic seizures in mice. J Neurochem (2009) 0.80

Myelination in the absence of UDP-galactose:ceramide galactosyl-transferase and fatty acid 2 -hydroxylase. BMC Neurosci (2011) 0.78

Diagnosis of alpha-mannosidosis by measuring alpha-mannosidase in plasma. Clin Chem (1992) 0.77

Human chorionic beta-mannosidase: comparison with beta-mannosidase from human cultured fibroblasts. Prenat Diagn (1992) 0.76

"Banana bodies" in disseminated lipogranulomatosis (Farber's disease). Am J Dermatopathol (1983) 0.76

Tissue accumulation of sulfatide and GM3 ganglioside in a patient with variant Farber disease. Clin Chim Acta (1995) 0.76

Articles by these authors

Fission and selective fusion govern mitochondrial segregation and elimination by autophagy. EMBO J (2008) 12.89

Molecular anatomy of a trafficking organelle. Cell (2006) 11.47

Ceramide triggers budding of exosome vesicles into multivesicular endosomes. Science (2008) 9.42

Cardiotoxicity of the cancer therapeutic agent imatinib mesylate. Nat Med (2006) 9.07

A comprehensive classification system for lipids. J Lipid Res (2005) 6.52

LMSD: LIPID MAPS structure database. Nucleic Acids Res (2006) 6.21

Lipidomics reveals a remarkable diversity of lipids in human plasma. J Lipid Res (2010) 4.14

Sphingolipidomics: high-throughput, structure-specific, and quantitative analysis of sphingolipids by liquid chromatography tandem mass spectrometry. Methods (2005) 3.42

Distinct epidermal growth factor receptor and KRAS mutation patterns in non-small cell lung cancer patients with different tobacco exposure and clinicopathologic features. Clin Cancer Res (2006) 3.40

Lasting blood-brain barrier disruption induces epileptic focus in the rat somatosensory cortex. J Neurosci (2004) 3.17

Ceramides and other bioactive sphingolipid backbones in health and disease: lipidomic analysis, metabolism and roles in membrane structure, dynamics, signaling and autophagy. Biochim Biophys Acta (2006) 3.10

Caspase-8 serves both apoptotic and nonapoptotic roles. J Immunol (2004) 3.09

When do Lasses (longevity assurance genes) become CerS (ceramide synthases)?: Insights into the regulation of ceramide synthesis. J Biol Chem (2006) 3.09

Imagable 4T1 model for the study of late stage breast cancer. BMC Cancer (2008) 3.03

The Runx3 transcription factor regulates development and survival of TrkC dorsal root ganglia neurons. EMBO J (2002) 2.99

Ceramide content is increased in skeletal muscle from obese insulin-resistant humans. Diabetes (2004) 2.86

Runx3 and Runx1 are required for CD8 T cell development during thymopoiesis. Proc Natl Acad Sci U S A (2003) 2.85

TGF-beta receptor-mediated albumin uptake into astrocytes is involved in neocortical epileptogenesis. Brain (2006) 2.80

SphK1 and SphK2, sphingosine kinase isoenzymes with opposing functions in sphingolipid metabolism. J Biol Chem (2005) 2.64

Quantitative analysis of sphingolipids for lipidomics using triple quadrupole and quadrupole linear ion trap mass spectrometers. J Lipid Res (2008) 2.62

Blood-brain barrier breakdown as a therapeutic target in traumatic brain injury. Nat Rev Neurol (2010) 2.56

Induction of sharp wave-ripple complexes in vitro and reorganization of hippocampal networks. Nat Neurosci (2005) 2.56

Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate. J Biol Chem (2007) 2.50

Regulated accumulation of desmosterol integrates macrophage lipid metabolism and inflammatory responses. Cell (2012) 2.38

High cholesterol level is essential for myelin membrane growth. Nat Neurosci (2005) 2.34

Perivascular clusters of dendritic cells provide critical survival signals to B cells in bone marrow niches. Nat Immunol (2008) 2.33

Axonal loss and neuroinflammation caused by peroxisome-deficient oligodendrocytes. Nat Genet (2007) 2.30

The H syndrome: a genodermatosis characterized by indurated, hyperpigmented, and hypertrichotic skin with systemic manifestations. J Am Acad Dermatol (2008) 2.29

The genetic interactome of prohibitins: coordinated control of cardiolipin and phosphatidylethanolamine by conserved regulators in mitochondria. J Cell Biol (2009) 2.22

A mouse macrophage lipidome. J Biol Chem (2010) 2.14

Neurocysticercosis: A natural human model of epileptogenesis. Epilepsia (2014) 2.11

Glucosylceramide synthase is an essential regulator of pathogenicity of Cryptococcus neoformans. J Clin Invest (2006) 2.08

Two mammalian longevity assurance gene (LAG1) family members, trh1 and trh4, regulate dihydroceramide synthesis using different fatty acyl-CoA donors. J Biol Chem (2003) 2.06

Biochemical and morphological properties of hepatitis C virus particles and determination of their lipidome. J Biol Chem (2010) 2.05

Lack of conventional dendritic cells is compatible with normal development and T cell homeostasis, but causes myeloid proliferative syndrome. Immunity (2008) 2.04

Upstream of growth and differentiation factor 1 (uog1), a mammalian homolog of the yeast longevity assurance gene 1 (LAG1), regulates N-stearoyl-sphinganine (C18-(dihydro)ceramide) synthesis in a fumonisin B1-independent manner in mammalian cells. J Biol Chem (2002) 2.03

Biodiversity of sphingoid bases ("sphingosines") and related amino alcohols. J Lipid Res (2008) 2.03

Aging up-regulates expression of inflammatory mediators in mouse adipose tissue. J Immunol (2007) 1.99

Mammalian ceramide synthases. IUBMB Life (2010) 1.94

Mucolipidosis II is caused by mutations in GNPTA encoding the alpha/beta GlcNAc-1-phosphotransferase. Nat Med (2005) 1.93

The structures of COPI-coated vesicles reveal alternate coatomer conformations and interactions. Science (2012) 1.90

Runx3 regulates mouse TGF-beta-mediated dendritic cell function and its absence results in airway inflammation. EMBO J (2004) 1.90

Common and uncommon pathogenic cascades in lysosomal storage diseases. J Biol Chem (2010) 1.85

Transcriptome profiling reveals TGF-beta signaling involvement in epileptogenesis. J Neurosci (2009) 1.85

Production of glucocerebrosidase with terminal mannose glycans for enzyme replacement therapy of Gaucher's disease using a plant cell system. Plant Biotechnol J (2007) 1.82

Adaptor protein complex 4 deficiency causes severe autosomal-recessive intellectual disability, progressive spastic paraplegia, shy character, and short stature. Am J Hum Genet (2011) 1.70

X-ray structure of human acid-beta-glucosidase, the defective enzyme in Gaucher disease. EMBO Rep (2003) 1.70

Ceramide synthase inhibition by fumonisin B1 causes accumulation of 1-deoxysphinganine: a novel category of bioactive 1-deoxysphingoid bases and 1-deoxydihydroceramides biosynthesized by mammalian cell lines and animals. J Biol Chem (2008) 1.69

A novel day blindness in sheep: epidemiological, behavioural, electrophysiological and histopathological studies. Vet J (2009) 1.69

Imaging MALDI mass spectrometry using an oscillating capillary nebulizer matrix coating system and its application to analysis of lipids in brain from a mouse model of Tay-Sachs/Sandhoff disease. Anal Chem (2008) 1.69

Caspase-8 deficiency in epidermal keratinocytes triggers an inflammatory skin disease. J Exp Med (2009) 1.69

Motor inhibition and learning impairments in school-aged children following exposure to organophosphate pesticides in infancy. Pediatr Res (2006) 1.66

Gaucher disease: pathological mechanisms and modern management. Br J Haematol (2005) 1.65

Fumonisins disrupt sphingolipid metabolism, folate transport, and neural tube development in embryo culture and in vivo: a potential risk factor for human neural tube defects among populations consuming fumonisin-contaminated maize. J Nutr (2004) 1.64

Structure-specific, quantitative methods for analysis of sphingolipids by liquid chromatography-tandem mass spectrometry: "inside-out" sphingolipidomics. Methods Enzymol (2007) 1.63

Membrane curvature induced by Arf1-GTP is essential for vesicle formation. Proc Natl Acad Sci U S A (2008) 1.63

A study of the clinical and radiological features in a cohort of 93 patients with a COL2A1 mutation causing spondyloepiphyseal dysplasia congenita or a related phenotype. Am J Med Genet A (2015) 1.60

Mutant p53 facilitates somatic cell reprogramming and augments the malignant potential of reprogrammed cells. J Exp Med (2010) 1.60

The molecular mechanism underlying Roberts syndrome involves loss of ESCO2 acetyltransferase activity. Hum Mol Genet (2008) 1.58

Quantitation of fatty acyl-coenzyme As in mammalian cells by liquid chromatography-electrospray ionization tandem mass spectrometry. J Lipid Res (2008) 1.57

Astrocytic dysfunction in epileptogenesis: consequence of altered potassium and glutamate homeostasis? J Neurosci (2009) 1.57

A critical role for ceramide synthase 2 in liver homeostasis: I. alterations in lipid metabolic pathways. J Biol Chem (2010) 1.56

The Thr224Asn mutation in the VPS45 gene is associated with the congenital neutropenia and primary myelofibrosis of infancy. Blood (2013) 1.55

The Sur7p family defines novel cortical domains in Saccharomyces cerevisiae, affects sphingolipid metabolism, and is involved in sporulation. Mol Cell Biol (2002) 1.54

Non-infectious aortitis: an important cause of severe aortic regurgitation. Asian Cardiovasc Thorac Ann (2006) 1.52

Combined tetanus, diphtheria, and 5-component pertussis vaccine for use in adolescents and adults. JAMA (2005) 1.51

Chronic cholinergic imbalances promote brain diffusion and transport abnormalities. FASEB J (2005) 1.50

Exposure to fumonisins and the occurrence of neural tube defects along the Texas-Mexico border. Environ Health Perspect (2006) 1.47

Subcellular organelle lipidomics in TLR-4-activated macrophages. J Lipid Res (2010) 1.46

Focal cortical dysfunction and blood-brain barrier disruption in patients with Postconcussion syndrome. J Clin Neurophysiol (2005) 1.46

Null leukemia inhibitory factor receptor (LIFR) mutations in Stuve-Wiedemann/Schwartz-Jampel type 2 syndrome. Am J Hum Genet (2004) 1.45

Molecular recognition of a single sphingolipid species by a protein's transmembrane domain. Nature (2012) 1.42

A critical role for ceramide synthase 2 in liver homeostasis: II. insights into molecular changes leading to hepatopathy. J Biol Chem (2010) 1.41