Published in J Neurochem on May 01, 1985
Isoflurane posttreatment reduces neonatal hypoxic-ischemic brain injury in rats by the sphingosine-1-phosphate/phosphatidylinositol-3-kinase/Akt pathway. Stroke (2010) 2.30
Occurrence of unusual molecular species of sphingomyelin containing 28-34-carbon polyenoic fatty acids in ram spermatozoa. Biochem J (1987) 1.00
Synergism of carbenicillin and gentamicin against enterococci. Antimicrob Agents Chemother (1973) 2.60
Heteroplasmic mtDNA mutation (T----G) at 8993 can cause Leigh disease when the percentage of abnormal mtDNA is high. Am J Hum Genet (1992) 2.41
Comparative pharmacokinetics of amikacin and kanamycin. Clin Pharmacol Ther (1974) 2.15
Inactivation of cefazolin, cephaloridine, and cephalothin by methicillin-sensitive and methicillin-resistant strains of Staphylococcus aureus. J Infect Dis (1975) 1.94
Mortality and cause of death in mucopolysaccharidosis type II-a historical review based on data from the Hunter Outcome Survey (HOS). J Inherit Metab Dis (2009) 1.89
Screening for carriers of Tay-Sachs disease among Ashkenazi Jews. A comparison of DNA-based and enzyme-based tests. N Engl J Med (1990) 1.78
Pancreatitis in patients with organic acidemias. J Pediatr (1994) 1.73
Postanesthetic apnea in full-term infants after pyloromyotomy. Anesthesiology (1994) 1.67
Copper-histidine therapy for Menkes disease. J Pediatr (1993) 1.56
Prostaglandin biosynthesis can be triggered by lipid peroxides. Arch Biochem Biophys (1979) 1.47
Sequence of DNA flanking the exons of the HEXA gene, and identification of mutations in Tay-Sachs disease. Am J Hum Genet (1991) 1.44
A simple method for the maintenance of oxygen saturation following intravenous induction of anaesthesia with propofol. Anaesthesia (1994) 1.39
The conversion of phenylalanine to tyrosine in man. Direct measurement by continuous intravenous tracer infusions of L-[ring-2H5]phenylalanine and L-[1-13C] tyrosine in the postabsorptive state. Metabolism (1982) 1.38
Enzyme replacement therapy by renal allotransplantation in Fabry's disease. N Engl J Med (1972) 1.38
Ticarcillin vs carbenicillin: clinical pharmacokinetics. Clin Pharmacol Ther (1975) 1.37
Early treatment of Menkes disease with parenteral copper-histidine: long-term follow-up of four treated patients. Am J Med Genet (1998) 1.31
Barth syndrome: clinical observations and genetic linkage studies. Am J Med Genet (1994) 1.31
Solar wind dynamic pressure and electric field as the main factors controlling Saturn's aurorae. Nature (2005) 1.30
Arteriopathy and coarctation of the abdominal aorta in children with mucopolysaccharidosis: imaging findings. AJR Am J Roentgenol (1991) 1.29
Pharmacokinetics of parenteral sodium cephalexin in comparison with cephalothin and cefazolin. Infection (1974) 1.28
Clinical pharmacology of amikacin and kanamycin. J Infect Dis (1976) 1.26
Pharmacokinetics of metronidazole as determined by bioassay. Antimicrob Agents Chemother (1974) 1.21
Altered regulation of cholesterol and cholesteryl ester synthesis in Chinese-hamster ovary cells overexpressing the oxysterol-binding protein is dependent on the pleckstrin homology domain. Biochem J (1997) 1.19
Differences in the metabolism of inositol and phosphoinositides by cultured cells of neuronal and glial origin. Biochim Biophys Acta (1989) 1.13
Chinese hamster ovary cells overexpressing the oxysterol binding protein (OSBP) display enhanced synthesis of sphingomyelin in response to 25-hydroxycholesterol. J Lipid Res (1999) 1.11
An Earth-like correspondence between Saturn's auroral features and radio emission. Nature (2005) 1.10
High-level gentamicin resistance in Enterococcus faecalis bacteremia. J Infect Dis (1991) 1.07
Ornithine transcarbamylase deficiency in females: an often overlooked cause of treatable encephalopathy. J Child Neurol (1995) 1.07
Funding crisis of the national health research and development program. Can Med Assoc J (1979) 1.07
Phosphatidylcholine biosynthesis in cultured glioma cells: evidence for channeling of intermediates. Biochim Biophys Acta (1989) 1.07
Differential effects of sphingomyelin hydrolysis and cholesterol transport on oxysterol-binding protein phosphorylation and Golgi localization. J Biol Chem (1998) 1.06
Sphingomyelinase activity at pH 7.4 in human brain and a comparison to activity at pH 5.0. J Lipid Res (1976) 1.05
Early copper-histidine treatment for Menkes disease. Nat Genet (1996) 1.05
Ceramide trihexosidosis (fabry's disease) without skin lesions. N Engl J Med (1971) 1.05
The effect of precursors, products, and product analogs of prostaglandin cyclooxygenase upon iris sphincter muscle. Life Sci (1978) 1.04
Membrane-bound CTP:phosphocholine cytidylyltransferase regulates the rate of phosphatidylcholine synthesis in HeLa cells treated with unsaturated fatty acids. Biochim Biophys Acta (1984) 1.04
Characterization of beta-galactosidase mutations Asp332-->Asn and Arg148-->Ser, and a polymorphism, Ser532-->Gly, in a case of GM1 gangliosidosis. Biochem J (2000) 1.02
A new Zn2+-stimulated sphingomyelinase in fetal bovine serum. J Biol Chem (1989) 1.01
Regulation of low density lipoprotein receptor and 3-hydroxy-3-methyl-glutaryl-CoA reductase activities are differentially affected in Niemann-Pick type C and type D fibroblasts. Biochem Cell Biol (1994) 1.01
Treatment of familial hypercholesterolemia in children and adolescents: effect of lovastatin. Canadian Lovastatin in Children Study Group. Pediatrics (1996) 1.01
Maternal phenylketonuria (PKU)--a review. Clin Biochem (1987) 1.00
Evidence that neutral sphingomyelinase of cultured murine neuroblastoma cells is oriented externally on the plasma membrane. Biochim Biophys Acta (1984) 0.99
Ornithine transcarbamylase deficiency presenting with strokelike episodes. J Pediatr (1993) 0.99
Heterozygote detection in angiokeratoma corporis diffusum (Anderson-Fabry disease). Studies on plasma, leucocytes, and hair follicles. J Med Genet (1977) 0.98
Autosomal recessive inheritance of vasopressin-resistant diabetes insipidus. Am J Med Genet (1991) 0.98
A mutation common in non-Jewish Tay-Sachs disease: frequency and RNA studies. Hum Mutat (1992) 0.98
Amerindian pyruvate carboxylase deficiency is associated with two distinct missense mutations. Am J Hum Genet (1998) 0.96
Regulation of eukaryotic phospholipid metabolism. FASEB J (1991) 0.96
Golgi localization and phosphorylation of oxysterol binding protein in Niemann-Pick C and U18666A-treated cells. J Lipid Res (2001) 0.95
Lysophosphatidylcholine as an intermediate in phosphatidylcholine metabolism and glycerophosphocholine synthesis in cultured cells: an evaluation of the roles of 1-acyl- and 2-acyl-lysophosphatidylcholine. Biochim Biophys Acta (1989) 0.94
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency in muscle: a new cause for recurrent myoglobinuria and encephalopathy. Ann Neurol (1991) 0.94
Vitamin B12 deficiency in adolescents and young adults with phenylketonuria. Lancet (1993) 0.93
Joubert syndrome with congenital hepatic fibrosis: an entity in the spectrum of oculo-encephalo-hepato-renal disorders. Am J Med Genet (1994) 0.93
U18666A inhibits intracellular cholesterol transport and neurotransmitter release in human neuroblastoma cells. Neurochem Res (1999) 0.91
Defective activity of acyl-CoA:cholesterol O-acyltransferase in Niemann-Pick type C and type D fibroblasts. Biochem J (1989) 0.91
Late-onset Tay-Sachs disease presenting as catatonic schizophrenia: diagnostic and treatment issues. J Clin Psychiatry (1995) 0.91
Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism (1998) 0.91
Pulmonary infiltration in Niemann-Pick disease type C. J Inherit Metab Dis (1996) 0.90
Evolution of the neuroimaging changes in fucosidosis type II. J Inherit Metab Dis (1996) 0.89
Acid and alkaline ceramidases of rat tissues. Biochem Cell Biol (1986) 0.89
Vitamin B12 deficiency in adolescents and young adults with phenylketonuria. Eur J Pediatr (1996) 0.89
Phosphatidylcholine metabolism in cultured cells: catabolism via glycerophosphocholine. Biochim Biophys Acta (1988) 0.89
The effect of vincristine on skeletal muscle in the rat. A correlative histochemical, ultrastructural and chemical study. J Neuropathol Exp Neurol (1972) 0.89
Glaucoma as an early complication of Hurler's disease. Arch Dis Child (1988) 0.89
Neutral and acid sphingomyelinases: somatotopographical distribution in human brain and distribution in rat organs. A possible relationship with the dopamine system. Brain Res (1979) 0.89
Failure to correct the metabolic defect by renal allotransplantion in Fabry's disease. Ann Intern Med (1976) 0.89
Alternate pathways in the desaturation and chain elongation of linolenic acid, 18:3(n-3), in cultured glioma cells. J Lipid Res (1991) 0.89
Homozygous presence of the crossover (fusion gene) mutation identified in a type II Gaucher disease fetus: is this analogous to the Gaucher knock-out mouse model? Biochem Med Metab Biol (1994) 0.88
Clathrin light chains are calcium-binding proteins. J Biol Chem (1987) 0.88
Gangliosides in developing rat brain. Isolation and composition of subcellular membranes enriched in gangliosides. Can J Biochem (1967) 0.88
Angiokeratoma corporis diffusum (Anderson-Fabry disease) in a single large family in Nova Scotia. J Med Genet (1978) 0.87
Serine utilization as a precursor of phosphatidylserine and alkenyl-(plasmenyl)-, alkyl-, and acylethanolamine phosphoglycerides in cultured glioma cells. J Biol Chem (1991) 0.87
A new variant of Sandhoff's disease. Pediatr Res (1974) 0.87
Pathways of sphingomyelin metabolism in cultured fibroblasts from normal and sphingomyelin lipidosis subjects. J Biol Chem (1983) 0.87
Identification and expression of eight novel mutations among non-Jewish patients with Canavan disease. Am J Hum Genet (1996) 0.87
Feto-maternal plasma phenylalanine concentration gradient from 19 weeks gestation to term. Clin Chim Acta (1994) 0.87
Neutral glycosphingolipids of serum lipoproteins in Fabry's disease. Biochim Biophys Acta (1976) 0.86
In vitro formation of polyunsaturated fatty acids by desaturation in rat brain: some properties of the enzymes in developing brain and comparisons with liver. J Neurochem (1978) 0.86
Experience with adolescents with phenylketonuria returned to phenylalanine-restricted diets. J Am Diet Assoc (1986) 0.86
"Hypotyrosinemia" in phenylketonuria. Mol Genet Metab (2000) 0.86
Decreased phosphatidylcholine biosynthesis and abnormal distribution of CTP:phosphocholine cytidylyltransferase in cholesterol auxotrophic Chinese hamster ovary cells. J Lipid Res (1997) 0.86
Salmonella arizonae bacteremia as the presenting manifestation of human immunodeficiency virus infection following rattlesnake meat ingestion. Rev Infect Dis (1990) 0.86
Enzyme replacement therapy for Gaucher's disease: the early Canadian experience. CMAJ (1998) 0.86
A pulsating auroral X-ray hot spot on Jupiter. Nature (2002) 0.86
An auroral flare at Jupiter. Nature (2001) 0.85
Rapid isolation of neuroblastoma plasma membranes on Percoll gradients. Characterization and lipid composition. Biochim Biophys Acta (1985) 0.85