Published in Biochim Biophys Acta on March 23, 1983
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Enzyme replacement therapy by renal allotransplantation in Fabry's disease. N Engl J Med (1972) 1.38
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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
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Ornithine transcarbamylase deficiency in females: an often overlooked cause of treatable encephalopathy. J Child Neurol (1995) 1.07
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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
Ceramide trihexosidosis (fabry's disease) without skin lesions. N Engl J Med (1971) 1.05
Early copper-histidine treatment for Menkes disease. Nat Genet (1996) 1.05
The effect of precursors, products, and product analogs of prostaglandin cyclooxygenase upon iris sphincter muscle. Life Sci (1978) 1.04
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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
Ornithine transcarbamylase deficiency presenting with strokelike episodes. J Pediatr (1993) 0.99
Evidence that neutral sphingomyelinase of cultured murine neuroblastoma cells is oriented externally on the plasma membrane. Biochim Biophys Acta (1984) 0.99
Autosomal recessive inheritance of vasopressin-resistant diabetes insipidus. Am J Med Genet (1991) 0.98
Heterozygote detection in angiokeratoma corporis diffusum (Anderson-Fabry disease). Studies on plasma, leucocytes, and hair follicles. J Med Genet (1977) 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
Role of glutamine synthetase in nitrogen metabolite repression in Aspergillus nidulans. J Bacteriol (2001) 0.94
Humoral hypercalcemia of malignancy: severe combined immunodeficient/beige mouse model of adult T-cell lymphoma independent of human T-cell lymphotropic virus type-1 tax expression. Am J Pathol (2001) 0.93
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
Catabolism of exogenous and endogenous sphingomyelin and phosphatidylcholine by homogenates and subcellular fractions of cultured neuroblastoma cells. Effects of anesthetics. J Neurochem (1985) 0.91
Late-onset Tay-Sachs disease presenting as catatonic schizophrenia: diagnostic and treatment issues. J Clin Psychiatry (1995) 0.91
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
Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism (1998) 0.91
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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
Glaucoma as an early complication of Hurler's disease. Arch Dis Child (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
Phosphatidylcholine metabolism in cultured cells: catabolism via glycerophosphocholine. Biochim Biophys Acta (1988) 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
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
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
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
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
Partial purification of a mannosyltransferase involved in the O-mannosylation of glycoproteins from Saccharomyces cerevisiae. Glycobiology (1991) 0.87
Feto-maternal plasma phenylalanine concentration gradient from 19 weeks gestation to term. Clin Chim Acta (1994) 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