Sphingosylphosphorylcholine in Niemann-Pick disease brain: accumulation in type A but not in type B.

Lysosphingolipids Inhibit Protein Kinase C: Implications for the Sphingolipidoses

1987 • 593 citations

Acid sphingomyelinase deficient mice: a model of types A and B Niemann–Pick disease

1995 • 469 citations

Intracellular Calcium Release Mediated by Sphingosine Derivatives Generated in Cells

1990 • 391 citations

Sphingomyelin synthesis in rat liver occurs predominantly at the cis and medial cisternae of the Golgi apparatus.

1990 • 369 citations

Quantitation of free sphingosine in liver by high-performance liquid chromatography

1988 • 315 citations

Krabbe disease: a galactosylsphingosine (psychosine) lipidosis.

1980 • 310 citations

Partial Enzyme Deficiencies: Residual Activities and the Development of Neurological Disorders

1983 • 273 citations

Twenty five years of the "psychosine hypothesis": a personal perspective of its history and present status.

1998 • 272 citations

Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase

1972 • 272 citations

Accumulation of Glucosylceramide and Glucosylsphingosine (Psychosine) in Cerebrum and Cerebellum in Infantile and Juvenile Gaucher Disease

1982 • 271 citations

Progressive Accumulation of Toxic Metabolite in a Genetic Leukodystrophy

1984 • 225 citations

THE DISTRIBUTION OF LIPIDS IN THE HUMAN NERVOUS SYSTEM—I

1964 • 194 citations

[Niemann-Pick disease [type A and B] (acid sphingomyelinase deficiencies)].

1998 • 173 citations

The distribution of lipids in the human nervous system. II. Lipid composition of human fetal and infant brain

1972 • 149 citations

Acid sphingomyelinase from human urine: purification and characterization

1987 • 131 citations

Signaling pathways for sphingosylphosphorylcholine-mediated mitogenesis in Swiss 3T3 fibroblasts.

1993 • 117 citations

Sphingosylphosphorylcholine is a remarkably potent mitogen for a variety of cell lines

1991 • 109 citations

Infantile Niemann-Pick Disease

1969 • 105 citations

Free sphingoid bases in tissues from patients with type C Niemann-Pick disease and other lysosomal storage disorders

1994 • 83 citations

THE DISTRIBUTION OF LIPIDS IN THE HUMAN NERVOUS SYSTEM‐V. GANGLIOSIDES AND ALLIED NEUTRAL GLYCOLIPIDS OF INFANT BRAIN

1973 • 77 citations

Acid Sphingomyelinase of Human Brain: Purification to Homogeneity

1982 • 75 citations

Accurate Differentiation of Neuronopathic and Nonneuronopathic Forms of Niemann‐Pick Disease by Evaluation of the Effective Residual Lysosomal Sphingomyelinase Activity in Intact Cells

1994 • 72 citations

The isolation and characterization of sphingomyelinase from human placental tissue

1977 • 70 citations

Studies in vitro on the Biosynthesis of Ceramide and Sphingomyelin A Reevaluation of Proposed Pathways

1980 • 67 citations

Deletion of arginine (608) in acid sphingomyelinase is the prevalent mutation among Niemann-Pick disease type B patients from northern Africa

1993 • 67 citations

Occurrence of Lysoganglioside Lyso-G_M2(II³-Neu5Ac-Gangliotriaosylsphingosine) in G_M2Gangliosidosis Brain

1986 • 66 citations

Infantile and fetal globoid cell leukodystrophy: Analysis of galactosylceramide and galactosylsphingosine

1988 • 61 citations

Composition of Gangliosides and Neutral Glycosphingolipids of Brain in Classical Tay‐Sachs and Sandhoff Disease: More Lyso‐GM2 in Sandhoff Disease?

1987 • 60 citations

Biochemical studies in Niemann‐Pick disease. III: In vitro and in vivo assays of sphingomyelin degradation in cultured skin fibroblasts and amniotic fluid cells for the diagnosis of the various forms of the disease

1985 • 59 citations

The distribution of lipids in the human nervous system. III. Fatty acid composition of phosphoglycerides of human foetal and infant brain

1973 • 56 citations

Chemical Pathology of Krabbe Disease: The Occurrence of Psychosine and Other Neutral Sphingoglycolipids

1976 • 56 citations

Lysosulfatide (Galactosylsphingosine‐3‐O‐Sulfate) from Metachromatic Leukodystrophy and Normal Human Brain

1989 • 54 citations

The phosphorylcholine acceptor in the phosphatidylcholine: ceramide cholinephosphotransferase reaction is the enzyme a transferase or a hydrolase?

1984 • 52 citations

Lysosulfatide (Sulfogalactosylsphingosine) Accumulation in Tissues from Patients with Metachromatic Leukodystrophy

1990 • 47 citations

Accumulation of Lysosphingolipids in Tissues from Patients with GM1 and GM2 Gangliosidoses

1992 • 43 citations

Sphingosylphosphocholine, a signaling molecule which accumulates in Niemann-Pick disease type A, stimulates DNA-binding activity of the transcription activator protein AP-1.

1995 • 41 citations

Juvenile dystonic lipidosis (variant of Niemann-Pick disease type C)

1984 • 35 citations

Peripheral neuropathy in type A Niemann-Pick disease

1984 • 33 citations

Modulation of protein kinase C by endogenous sphingosine: inhibition of phorbol dibutyrate binding in Niemann–Pick C fibroblasts

1997 • 33 citations

Altered levels of tissue glycoproteins, gangliosides, glycosaminoglycans and lipids in niemann-pick's disease

1973 • 32 citations

Lysosphingolipids and mitochondrial function. II. Deleterious effects of sphingosylphosphorylcholine

1988 • 29 citations

Enzyme activities and phospholipid storage patterns in brain and spleen samples from niemann‐pick disease variants: a comparison of neuropathic and non‐neuropathic forms

1985 • 28 citations

Fatal Liver Failure in Two Children with Niemann-Pick Disease Type B

1991 • 27 citations

Studies on the hydrophobic properties of sphingomyelinase

1981 • 26 citations

Enzymic synthesis of sphingosylphosphorylcholine

1968 • 24 citations

The potent lipid mitogen sphingosylphosphocholine activates the DNA binding activity of upstream stimulating factor (USF), a basic helix-loop-helix-zipper protein

1998 • 21 citations

Transgenic introduction of human galactosylceramidase into twitcher mouse: Significant phenotype improvement with a minimal expression

1997 • 18 citations

Metabolism and Neurite Promoting Effect of Exogenous Sphingosylphosphocholine in Cultured Murine Neuroblastoma Cells1

1993 • 12 citations

Sphingomyelinase and Niemann-Pick Disease

1986 • 10 citations

[Preparative production of sphingosinephosphorvlcholine].

1961 • 9 citations

Psychosine and Sphingosylphosphorylcholine Bind to Mitochondrial Membranes and Disrupt their Function

1988 • 5 citations