Biosynthesis and metabolic degradation of sphingolipids not containing sialic acid

Enzymatic Defect in Fabry's Disease

1967 • 1,035 citations

Metabolism of glucocerebrosides II. Evidence of an enzymatic deficiency in Gaucher's disease

1965 • 800 citations

Tay-Sachs Disease: Generalized Absence of a Beta-D- N -Acetylhexosaminidase Component

1969 • 707 citations

Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin

1965 • 471 citations

Fabry's Disease: Alpha-Galactosidase Deficiency

1970 • 450 citations

Globoid Cell Leucodystrophy (Krabbe's Disease): Deficiency of Galactocerebroside β-Galactosidase

1970 • 447 citations

The Structure and Function of Nervous Tissue

1969 • 429 citations

The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae.

1966 • 413 citations

Glycolipids of hamster fibroblasts and derived malignant-transformed cell lines.

1968 • 336 citations

QUAKING MOUSE: ISOLATION AND CHARACTERIZATION OF MYELIN PROTEIN

1971 • 305 citations

Sphingomyelinase in normal human spleens and in spleens from subjects with Niemann-Pick disease

1967 • 302 citations

Diagnosis of Gaucher's Disease and Niemann-Pick Disease with Small Samples of Venous Blood

1967 • 290 citations

Eine Cerebrosidsulfatase aus Schweineniere

1964 • 278 citations

The Metabolism of Glucocerebrosides

1965 • 276 citations

On the chemistry and occurrence of sphingolipid long-chain bases

1970 • 271 citations

The Metabolic Basis of Inherited Disease

1967 • 263 citations

Variation of β‐N‐acetylhexosaminidase‐pattern in Tay‐Sachs disease

1969 • 258 citations

The Metabolism of Sphingomyelin

1966 • 248 citations

Cerebroside 3-sulfate as a physiological substrate of arylsulfatase A

1968 • 237 citations

Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid

1969 • 231 citations

The chemical structure of normal human brain and Tay-Sachs gangliosides

1962 • 229 citations

Anomeric Structures of Globoside and Ceramide Trihexoside of Human Erythrocytes and Hamster Fibroblasts

1971 • 227 citations

Enzymatic Synthesis of Ceramide-Glucose and Ceramide-Lactose by Glycosyltransferases from Embryonic Chicken Brain

1968 • 215 citations

Evidence for the genetic block in metachromatic leucodystrophy (ML)

1965 • 209 citations

The presence of sphingomyelin- and ceramide-cleaving enzymes in the small intestinal tract

1969 • 207 citations

Organizational difference of cell surface “hematoside” in normal and virally transformed cells

1968 • 191 citations

Lactosylceramide galactosidase: Comparison with other sphingolipid hydrolases in developing rat brain

1969 • 189 citations

The fatty acid composition of brain sphingolipids: sphingomyelin, ceramide, cerebroside, and cerebroside sulfate

1964 • 188 citations

A CONTROLLED STUDY OF ENZYMIC ACTIVITIES IN THREE HUMAN DISORDERS OF GLYCOLIPID METABOLISM*

1963 • 186 citations

Changes in the fatty acid composition of cerebrosides and sulfatides of human nervous tissue with age

1968 • 183 citations

The Enzymatic Synthesis of Psychosine

1960 • 178 citations

The Enzymatic Synthesis of Sphingomyelin

1958 • 177 citations

Specificity in Ceramide Biosynthesis from Long Chain Bases and Various Fatty Acyl Coenzyme A's by Brain Microsomes

1970 • 176 citations

Immunochemical Reactions of Lipids

2015 • 174 citations

Inborn Disorders of Sphingolipid Metabolism

1967 • 158 citations

Enzymatic Hydrolysis of Sphingolipids

1966 • 158 citations

Gaucher Cells in Chronic Myelocytic Leukemia: An Acquired Abnormality

1969 • 156 citations

Isolation of β-galactosidase and β-glucosidase from brain

1966 • 149 citations

The Incorporation of Galactose into Galactolipides

1958 • 142 citations

THE IN VIVO AND IN VITRO SYNTHESIS OF SULPHATIDES DURING DEVELOPMENT*

1967 • 142 citations

Changes in sphingosine and fatty acid components of the gangliosides in developing rat and human brain

1966 • 137 citations

Structure of the Tay-Sachs' Ganglioside. I^*

1965 • 137 citations

The Enzymatic Synthesis of Sphingosine

1958 • 133 citations

Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease

1969 • 133 citations

The Metabolism of Glucocerebrosides

1965 • 126 citations

Fatty acid composition of human brain sphingomyelins: normal variation with age and changes during myelin disorders

1965 • 125 citations

In vivo conversions of cerebroside and ceramide in rat brain

1965 • 122 citations

The lysosomal localization of sphingolipid hydrolases

1968 • 122 citations

Developing rat brain: changes in cholesterol, galactolipids, and the individual fatty acids of gangliosides and glycerophosphatides

1965 • 121 citations

Enzymatic synthesis of ceramide

1966 • 120 citations

Quaking mouse: ultrastructural evidence for arrest of myelinogenesis

1971 • 115 citations

Synthesis of C18- and C20-Dihydrosphingosines, Ketodihydrosphingosines, and Ceramides by Microsomal Preparations from Mouse Brain

1970 • 114 citations

Cerebroside galactosidase of brain

1966 • 113 citations

The biosynthesis by brain microsomes of cerebrosides containing nonhydroxy fatty acids

1970 • 111 citations

Isolation of β-N-Acetylhexosaminidase, β-N-Acetylglucosaminidase, and β-N-Acetylgalactosaminidase from Calf Brain^*

1967 • 110 citations

Sphingolipids

1971 • 110 citations

Enzymic hydrolysis of sphingolipids. VIII. Purification and properties of rat brain ceramidase

1969 • 108 citations

Turnover of the fatty acids of rat brain gangliosides, glycerophosphatides, cerebrosides, and sulfatides as a function of age

1965 • 107 citations

Biosynthesis of Sphingolipid Bases

1968 • 106 citations

Glycolipids

1965 • 105 citations

Composition and Synthesis of Gangliosides in Rat Hepatocyte and Hepatoma Cell Lines

1969 • 103 citations

Synthesis of galactosyl ceramide and glucosyl ceramide by rat brain: Assay procedures and changes with age

1972 • 102 citations

Enzymatic Synthesis of Galactocerebroside by a Galactosyltransferase from Embryonic Chicken Brain

1971 • 102 citations

Metabolism of cerebroside in the intestinal tract of the rat

1969 • 100 citations

Composition of cerebroside acids as a function of age

1959 • 98 citations

Characterization of Cerebroside Sulfotransferase from Rat Brain

1971 • 96 citations

Sulfation of Glycosphingolipids and Related Carbohydrates by Brain Preparations from Young Rats

1968 • 94 citations

CEREBROSIDE GALACTOSIDASE: A METHOD FOR DETERMINATION AND A COMPARISON WITH OTHER LYSOSOMAL ENZYMES IN DEVELOPING RAT BRAIN¹

1969 • 94 citations

G _M2 ‐gangliosidosis with total hexosaminidase deficiency

1971 • 93 citations

Glycosphingolipids having blood-group ABH and Lewis specificities

1970 • 92 citations

Deficiency of sphingomyelin-cleaving enzyme activity in tissue cultures derived from patients with Niemann-Pick disease

1969 • 90 citations

The Metabolism of Ceramide Trihexosides

1967 • 90 citations

Studies on the Total Enzymatic Synthesis of Cerebrosides

1962 • 88 citations

Lactosyl Ceramidosis: Catabolic Enzyme Defect of Glycosphingolipid Metabolism

1970 • 88 citations

The Enzymatic Synthesis of Sphingosine

1958 • 86 citations

Identification of ceramide phosphorylethanolamine and ceramide phosphorylglycerol in the lipids of an anaerobic bacterium

1969 • 82 citations

Isotopic studies of the biosynthesis of the cerebroside fatty acids in rats

1963 • 82 citations

Metabolism of Sphingosine Bases, XIV. Sphinganine (Dihydrosphingosine), an Effective Donor of the Alk-1’-enyl Chain of Plasmalogens

1970 • 78 citations

Metabolism of Sphingosine Bases, V. Biosynthesis of Dihydrosphingosinein vitro

1968 • 74 citations

Fatty acids of human brain cerebrosides

1961 • 74 citations

Enzymic hydrolysis of sphingomyelin by rat liver

1966 • 72 citations

Die Leukodystrophie, Typ Scholz, (metachromatische Form der diffusen Sklerose) als Sphingolipoidose (Cerebrosid-schwefelsäureester-Speicherkrankheit)

1960 • 67 citations

Studies in globoid leukodystrophy: Enzymatic and lipid findings in the canine form

1970 • 66 citations

Metabolism of Sphingosine Bases, IX. Degradationin vitroof Dihydrosphingosine and Dihydrosphingosine phosphate to Palmitaldehyde and Ethanolamine phosphate

1968 • 66 citations

THE PRECURSORS OF SPHINGOSINE IN BRAIN TISSUE

1954 • 66 citations

Enzymic hydrolysis of sphingolipids. Hydrolysis of ceramide glucoside by an enzyme from ox brain

1966 • 65 citations

GLYCOSYL TRANSFERASES OF MICROSOMAL FRACTIONS FROM BRAIN: SYNTHESIS OF GLUCOSYL CERAMIDE AND GALACTOSYL CERAMIDE DURING DEVELOPMENT AND THE DISTRIBUTION OF GLUCOSE AND GALACTOSE TRANSFERASE IN WHITE AND GREY MATTER¹

1971 • 63 citations

Properties of cerebroside galactosidase

1968 • 63 citations

Characterization of the iso-branched sphinganines from the ceramide phospholipids of Bacteroides melaninogenicus

1969 • 62 citations

Metabolism of sulfatides. I. The effect of galactocerebrosides on the synthesis of sulfatides

1965 • 61 citations

An Alternative Pathway for the Enzymatic Synthesis of Sphingomyelin

1965 • 60 citations

Metabolism of Sphingosine Bases, VIII. Distribution, Isolation and Properties of D-3-Oxosphinganine Reductase. Stereospecificity of the NADPH-dependent Reduction Reaction of 3-Oxodihydrosphingosine(2-Amino-1-hydroxyoctadecane-3-one)

1968 • 60 citations

FORMATION AND TURNOVER OF THE MAJOR BRAIN GANGLIOSIDES DURING DEVELOPMENT *

1967 • 58 citations

Quaking mouse:In vitro studies of brain sphingolipid biosynthesis

1971 • 58 citations

Enzymatic Hydrolysis of Sphingolipids. VI. Hydrolysis of Ceramide Glycosides by Calf Brain β-N-Acetylhexosaminidase^*

1967 • 56 citations

Extracellular lipids of yeasts

1967 • 56 citations

Metabolism of Sphingosine Bases, XV. Enzymatic Degradation of 4t-Sphingenine 1-phosphate (Sphingosine 1-phosphate) to 2t-Hexadecen-1-al and Ethanolamine phosphate

1970 • 56 citations

Purification of cerebroside galactosidase from rat brain

1968 • 55 citations

Biosynthesis of Lactosylceramide and Triglycosylceramide by Galactosyltransferases from Rat Spleen

1969 • 54 citations

Studies on the Biosynthesis of Plasmalogens. Precursors in the Biosynthesis of Plasmalogens: On the Stereospecificity of the Biochemical Dehydrogenation of the 1-O-Alkyl Glyceryl to the 1-O-Alk-1’-enyl Glyceryl Ether Bond

1971 • 53 citations

Enzymic hydrolysis of sphingolipids. Hydrolysis of ceramide lactoside by an enzyme from rat brain

1966 • 52 citations

The metabolism of glyceride glycolipids. II. Biosynthesis of monogalactosyl diglyceride from uridine diphosphate galactose and diglyceride in brain

1968 • 52 citations

Formation of cerebroside sulphate from 3′-phosphoadenosine 5′-phosphosulphate in sheep brain

1965 • 51 citations

Biosynthesis of glycolipids: Incorporation of N‐acetyl galactosamine by a rat brain particulate preparation

1969 • 51 citations

Enzymatic Formation of Dihydrosphingosine 1-Phosphate

1970 • 50 citations

Lipid Metabolism

1970 • 50 citations

BIOSYNTHESIS OF LACTOSYLCERAMIDE BY RAT BRAIN PREPARATIONS AND COMPARISON WITH THE FORMATION OF GANGLIOSIDE G_M1 AND PSYCHOSINE DURING DEVELOPMENT

1970 • 49 citations

Chemistry and Metabolism of Sphingolipids. On the Biosynthesis of Phytosphingosine by Yeast^*

1967 • 49 citations

Structure of sulfatides biosynthesized in vitro

1971 • 48 citations

Investigation of the enzymatic synthesis of sphingomyelin

1968 • 48 citations

STUDIES ON THE BIOSYNTHESIS OF GANGLIOSIDES

1967 • 48 citations

Biosynthesis of Sphingolipid Bases

1969 • 48 citations

Studies on the production of sphingolipid bases by the yeast, Hansenula ciferri

1965 • 47 citations

THE REGIONAL DISTRIBUTION, AGE DEPENDENT VARIATION AND SPECIES DIFFERENCES OF BRAIN ARYLSULPHATASES

1971 • 47 citations

The enzymic synthesis of ganglioside: I. Brain uridine diphopphate D‐galactose: N‐acetyl‐galactosaminyl‐galactosyl‐glucosyl‐ceramide galactosyl transferase

1969 • 46 citations

Biosynthesis of sphingosine and dihydrosphingosine by cell-free systems from Hansenula ciferri

1970 • 46 citations

Cytolipin H, a Pure Lipid Hapten isolated from Human Carcinoma

1958 • 45 citations

The biosynthesis of dihydrosphingosine in cell-free preparations of Hansenula ciferri.

1967 • 45 citations

Biosynthesis of sphingolipid bases

1971 • 44 citations

Structure of kidney ceramide dihexoside sulfate

1968 • 43 citations

Chemistry of sphingolipids

1970 • 41 citations

Glycosphingolipids of animal tissue

1970 • 41 citations

Sphingolipid metabolism in leukemic leukocytes.

1967 • 40 citations

Metabolism of Sphingosine Bases, II. Studies on the Degradation and Transformation of [3-¹⁴C]erythro-DL-Dihydrosphingosine, [7-³H]erythro-DL-Sphingosine, [5-³H]threo-L-Dihydrosphingosine and [3-¹⁴C;1-³H]erythro-DL-Dihydrosphingosine in Rat Liver

1967 • 40 citations

The Configuration of Sphingosine Synthesized in Rat Brain Homogenates

1962 • 38 citations

THE METABOLISM OF GANGLIOSIDES IN CEREBRAL LIPIDOSES

1967 • 38 citations

THE BIOSYNTHESIS OF SPHINGOSINE

1953 • 36 citations

The Biosynthesis of Sphingosine

1963 • 36 citations

Enzymic hydrolysis of sphingomyelins by a toxin of Corynebacterium ovis

1967 • 36 citations

A Cerebroside Sulphotransferase Deficiency in a Human Disorder of Myelin

1967 • 36 citations

The enzymatic synthesis of ceramide lactoside from ceramide glucoside and UDP-galactose

1967 • 35 citations

The utilization and degradation of phytosphingosine by rat liver

1967 • 35 citations

Metabolism of Sphingosine Bases, VI. Synthesis and Degradation of Sphingosine Bases inHansenula ciferrii

1968 • 35 citations

BIOSYNTHESIS OF MYELIN PROTEINS IN VITRO¹

1971 • 35 citations

Effect of vitamin B6 deficiency on the composition of yeast lipids

1965 • 34 citations

Fatty acid composition of brain glycolipids in Alzheimer's disease, senile dementia, and cerebrocortical atrophy

1968 • 34 citations

Stereochemistry of the Hydroxylation in 4-Hydroxysphinganine Formation and the Steric Course of Hydrogen Elimination in Sphing-4-enine Biosynthesis

1971 • 33 citations

Metabolism of Sphingosine Bases, IV. 2-Amino-1-hydroxyoctadecane-3-one (3-oxodihydrosphingosine), the Common Intermediate in the Biosynthesis of Dihydrosphingosine and Sphingosine and in the Degradation of Dihydrosphingosine

1967 • 33 citations

The biosynthesis of digalactosylceramide in the kidney of the C57BL mouse

1970 • 33 citations

THE EFFECTS OF INHIBITORS OF PROTEIN SYNTHESIS ON INCORPORATION OF LIPIDS INTO MYELIN¹²

1971 • 33 citations

Metabolism of labeled dihydrosphingomyelin in vivo

1968 • 32 citations

BIOSYNTHESIS OF CERAMIDE BY RAT BRAIN HOMOGENATES¹

1957 • 32 citations

The chemistry of the lipids of posthemolytic residue or stroma of erythrocytes. 8. Further studies on human red cell glycolipids.

1965 • 32 citations

Enzymatic synthesis of psychosine in “Jimpy” mice brain

1969 • 32 citations

The enzymatic phosphorylation of sphinganine

1969 • 32 citations

Über den enzymatischen Abbau von N-acetylneuraminsäure-freien Gangliosidresten (Ceramid-oligosacchariden)

1964 • 30 citations

Substrate Specificity for Ceramide in the Enzymatic Formation of Sphingomyelin

1968 • 29 citations

Cerebral Lipidoses

1970 • 29 citations

THE BIOSYNTHESIS OF SPHINGOSINE

1954 • 28 citations

Ceramide-like synthetic amides that inhibit cerebroside galactosidase

1972 • 27 citations

Chemistry of sphingolipids

1969 • 26 citations

Glycosphingolipids of animal tissue

1970 • 26 citations

Enzymatic deficiency in neurological mutants. Brain uridine diphosphate galactose: Ceramide galactosyl transferase in jimpy mouse

1970 • 26 citations

Response of sphingolipid hydrolases in spleen and liver to increased erythrocytorhexis

1967 • 26 citations

Sphingolipid composition of the avian salt gland

1969 • 24 citations

On the biosynthesis of cerebrosides containing non-hydroxy acids

1971 • 24 citations

Enzymic synthesis of sphingosylphosphorylcholine

1968 • 24 citations

Sphingolipid metabolism II. The biosynthesis of 3‐keto‐dihydrosphingosine by a partially‐purified enzyme from rat brain

1970 • 23 citations

The in vitro degradation of dihydrosphingosine

1969 • 23 citations

In,vivo conversion of erythro and threo DL-sphingosine-3H to ceramide and sphingomyelin

1966 • 23 citations

Biosynthesis of Phytosphingosine

1967 • 22 citations

The degradation of tritiated dihydrosphingosine in the intact rat

1968 • 21 citations

Metabolism of Sphingosine Bases, X. Degradation of [1-¹⁴C]Dihydrosphingosine (Sphinganine), [1-¹⁴C]2-Amino-1,3-dihydroxyheptane and [1-¹⁴C]Dihydrosphingosine phosphate im Rat Liver

1969 • 20 citations

On the biosynthesis of cerebrosides from 2-hydroxy acid ceramides: Use of deuterium labeled substrate and multiple ion detector

1970 • 20 citations

Zur Kenntnis der Gehirnceramide und der darin vorkommenden Sphingosinbasen

1968 • 19 citations

Degradation of sphingosine, dihydrosphingosine, and phytosphingosine in rats

1968 • 18 citations

Vitamin K stimulation of sphingolipid synthesis

1971 • 18 citations

Degradation of sphingosine bases by cell-free preparations. α-hydroxy palmitic acid, an intermediate of phytosphingosine degradation

1968 • 17 citations

Glycosphingolipid biosynthesis in kidneys of normal C3HHe mice and of those with BP8 ascites tumours

1970 • 17 citations

Stimulation and inhibition of sphingomyelin synthetase

1968 • 15 citations

The Relationship between Phospholipid Biosynthesis, Sphingosine Degradation and Serine Metabolism

1969 • 14 citations

New technique for enzymic hydrolysis of glycosphingolipids

1969 • 12 citations

Fatty acids of glycosphingolipids from pig blood fractions

1970 • 10 citations

On cerebrosides and gangliosides

1970 • 9 citations

Metabolism of Sphingosine Bases, XII. Eicosasphinganine (Eicosadihydrosphingosine) and 3-Dehydroeicosasphinganine (3-Dehydroeicosadihydrosphingosine)

1969 • 8 citations

Cerebrosides and Sulfatides

1970 • 4 citations

Sphingomyelin: Enzymatic Reactions

1970 • 3 citations

In Vivo Utilization of Dihydrosphingosine-4,5-3H

1968 • 1 citations