Published in J Histochem Cytochem on August 01, 1999
Signals from the lysosome: a control centre for cellular clearance and energy metabolism. Nat Rev Mol Cell Biol (2013) 3.13
Sorting of EGF and transferrin at the plasma membrane and by cargo-specific signaling to EEA1-enriched endosomes. J Cell Sci (2008) 1.70
Relationships between EGFR signaling-competent and endocytosis-competent membrane microdomains. Mol Biol Cell (2005) 1.67
Body-barrier surveillance by epidermal γδ TCRs. Nat Immunol (2012) 1.48
Mechanism of amyloid plaque formation suggests an intracellular basis of Abeta pathogenicity. Proc Natl Acad Sci U S A (2010) 1.42
Cholera toxin toxicity does not require functional Arf6- and dynamin-dependent endocytic pathways. Mol Biol Cell (2004) 1.32
Biosynthesis and degradation of mammalian glycosphingolipids. Philos Trans R Soc Lond B Biol Sci (2003) 1.29
Murine cytomegalovirus perturbs endosomal trafficking of major histocompatibility complex class I molecules in the early phase of infection. J Virol (2010) 0.85
My journey into the world of sphingolipids and sphingolipidoses. Proc Jpn Acad Ser B Phys Biol Sci (2012) 0.79
Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activity. J Lipid Res (2015) 0.78
Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase. J Lipid Res (2017) 0.75
Atypical juvenile presentation of GM2 gangliosidosis AB in a patient compound-heterozygote for c.259G > T and c.164C > T mutations in the GM2A gene. Mol Genet Metab Rep (2017) 0.75
Impact of GM1 on Membrane-mediated Aggregation/Oligomerization of β-amyloid: Unifying View. Biophys J (2017) 0.75
Establishment of a human cell line (Mono Mac 6) with characteristics of mature monocytes. Int J Cancer (1988) 3.66
Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease. Nat Genet (1995) 2.66
Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nat Genet (1995) 2.64
CD95 signaling via ceramide-rich membrane rafts. J Biol Chem (2001) 2.48
Luminal membrane retrieved after exocytosis reaches most golgi cisternae in secretory cells. Proc Natl Acad Sci U S A (1977) 2.33
Partial enzyme deficiencies: residual activities and the development of neurological disorders. Dev Neurosci (1984) 2.23
Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease. Hum Genet (1992) 2.12
Enzyme alterations and lipid storage in three variants of Tay-Sachs disease. J Neurochem (1971) 2.11
A vital role for glycosphingolipid synthesis during development and differentiation. Proc Natl Acad Sci U S A (1999) 2.05
The human acid ceramidase gene (ASAH): structure, chromosomal location, mutation analysis, and expression. Genomics (1999) 2.04
Quantifying lamella dynamics of cultured cells by SACED, a new computer-assisted motion analysis. Exp Cell Res (1999) 2.04
AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2. Proc Natl Acad Sci U S A (1978) 2.02
A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder. J Clin Invest (1999) 1.96
A new sensitive colorimetric assay for peroxidase using 3,3'-diaminobenzidine as hydrogen donor. Anal Biochem (1973) 1.96
Deficient hexozaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs. Life Sci (1968) 1.95
Cholera toxin is exported from microsomes by the Sec61p complex. J Cell Biol (2000) 1.90
Human acid sphingomyelinase. Isolation, nucleotide sequence and expression of the full-length and alternatively spliced cDNAs. J Biol Chem (1991) 1.89
Association of genotypes affecting the expression of interleukin-1beta or interleukin-1 receptor antagonist with osteoarthritis. Arthritis Rheum (2000) 1.88
Translocation arrest by reversible folding of a precursor protein imported into mitochondria. A means to quantitate translocation contact sites. J Cell Biol (1989) 1.84
[The localization of peroxidase and acid phosphatase in eosinophil leukocytes during maturation. Electron microscopical cytochemical studies on the bone marrow of the rat and rabbit]. Z Zellforsch Mikrosk Anat (1969) 1.79
Physiology and pathophysiology of sphingolipid metabolism and signaling. Biochim Biophys Acta (2000) 1.77
Diameter of the cell-to-cell junctional membrane channels as probed with neutral molecules. Science (1981) 1.76
Subcellular localization and membrane topology of serine palmitoyltransferase, 3-dehydrosphinganine reductase, and sphinganine N-acyltransferase in mouse liver. J Biol Chem (1992) 1.76
Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids. Hum Mol Genet (1996) 1.70
STED nanoscopy reveals molecular details of cholesterol- and cytoskeleton-modulated lipid interactions in living cells. Biophys J (2011) 1.69
Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A. J Biol Chem (1985) 1.66
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures. J Biol Chem (2000) 1.64
Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis. Nat Genet (1996) 1.63
Ganglioside metabolism. Enzymology, Topology, and regulation. J Biol Chem (1993) 1.59
Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts. EMBO J (1989) 1.55
Exocytosis in secretory cells of rat lacrimal gland. Peroxidase release from lobules and isolated cells upon cholinergic stimulation. J Cell Biol (1976) 1.50
Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. J Clin Invest (1998) 1.49
Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease. J Biol Chem (1996) 1.49
Lysosomal enzyme precursors in human fibroblasts. Activation of cathepsin D precursor in vitro and activity of beta-hexosaminidase A precursor towards ganglioside GM2. Eur J Biochem (1982) 1.48
Acidic sphingomyelinase mediates entry of N. gonorrhoeae into nonphagocytic cells. Cell (1997) 1.47
Activator proteins and topology of lysosomal sphingolipid catabolism. Biochim Biophys Acta (1992) 1.46
The tricyclic antidepressant desipramine causes proteolytic degradation of lysosomal sphingomyelinase in human fibroblasts. Biol Chem Hoppe Seyler (1994) 1.46
The coronary endothelium: a highly active metabolic barrier for adenosine. Basic Res Cardiol (1986) 1.45
[Enrichment and characterization of 2 forms of human N-acetyl-beta-D-hexosaminidase]. Hoppe Seylers Z Physiol Chem (1971) 1.45
GM2-gangliosidosis B1 variant: analysis of beta-hexosaminidase alpha gene abnormalities in seven patients. Am J Hum Genet (1990) 1.43
[Clinical aspects and therapy of amniotic fluid embolism. Illustration based on a case report]. Z Geburtshilfe Neonatol (1997) 1.39
Characterization of ceramide synthesis. A dihydroceramide desaturase introduces the 4,5-trans-double bond of sphingosine at the level of dihydroceramide. J Biol Chem (1997) 1.37
Characterization of translocation contact sites involved in the import of mitochondrial proteins. J Cell Biol (1987) 1.37
Ganglioside GM2 N-acetyl-beta-D-galactosaminidase activity in cultured fibroblasts of late-infantile and adult GM2 gangliosidosis patients and of healthy probands with low hexosaminidase level. Am J Hum Genet (1983) 1.36
Acid sphingomyelinase deficiency. Phenotype variability with prevalence of intermediate phenotype in a series of twenty-five Czech and Slovak patients. A multi-approach study. J Inherit Metab Dis (2005) 1.36
Metabolism and intracellular transport of glycosphingolipids. Biochemistry (1990) 1.34
Deficient hexosaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs. Pathol Eur (1968) 1.34
Sphingolipid activator proteins are required for epidermal permeability barrier formation. J Biol Chem (1999) 1.32
[The localization of endogenous peroxidase in the parotid gland of the rat]. Z Zellforsch Mikrosk Anat (1970) 1.32
Stimulation of acid sphingomyelinase activity by lysosomal lipids and sphingolipid activator proteins. Biol Chem (2001) 1.32
Membrane retrieval in epithelial cells of isolated thyroid follicles. Eur J Cell Biol (1979) 1.30
Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy. Am J Hum Genet (1981) 1.29
Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons. J Biol Chem (1999) 1.29
A simple and novel method for tritium labeling of gangliosides and other sphingolipids. Biochim Biophys Acta (1978) 1.27
Acid sphingomyelinase from human urine: purification and characterization. Biochim Biophys Acta (1987) 1.26
The localization of endogenous peroxidase in the lacrimal gland of the rat during postnatal development. Electron microscope cytochemical and biochemical studies. J Cell Biol (1972) 1.26
Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. J Inherit Metab Dis (2004) 1.26
Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease. FEBS Lett (1991) 1.26
Cellular uptake of saposin (SAP) precursor and lysosomal delivery by the low density lipoprotein receptor-related protein (LRP). EMBO J (1998) 1.25
Fumonisin B1 inhibits sphingosine (sphinganine) N-acyltransferase and de novo sphingolipid biosynthesis in cultured neurons in situ. J Biol Chem (1993) 1.25
Transcytosis in thyroid follicle cells. J Cell Biol (1983) 1.24
A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB. FEBS Lett (1991) 1.24
Lysogangliosides: synthesis and use in preparing labeled gangliosides. Methods Enzymol (1987) 1.24
Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A. Hoppe Seylers Z Physiol Chem (1979) 1.24
Purification, characterization, and biosynthesis of human acid ceramidase. J Biol Chem (1995) 1.23
Mice with type 2 and 3 Gaucher disease point mutations generated by a single insertion mutagenesis procedure. Proc Natl Acad Sci U S A (1998) 1.23
Specificity of human liver hexosaminidases A and B against glycosphingolipids GM2 and GA2. Purification of the enzymes by affinity chromatography employing specific elution. Hoppe Seylers Z Physiol Chem (1977) 1.20
Biochemistry and genetics of gangliosidoses. Hum Genet (1979) 1.20
Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators. J Biol Chem (1998) 1.20
Overexpression of acid ceramidase protects from tumor necrosis factor-induced cell death. J Exp Med (2000) 1.19
Incorporation and metabolism of ganglioside GM2 in skin fibroblasts from normal and GM2 gangliosidosis subjects. Eur J Biochem (1985) 1.16
Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2. Eur J Biochem (1982) 1.16
Incorporation of ganglioside analogues into fibroblast cell membranes. A spin-label study. Biochemistry (1983) 1.15
Cloning and characterization of the full-length cDNA and genomic sequences encoding murine acid ceramidase. Genomics (1998) 1.15
Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3. Neurobiol Dis (2003) 1.15
Occurrence of two molecular forms of human acid sphingomyelinase. Biochem J (1994) 1.15
Cathepsin K in thyroid epithelial cells: sequence, localization and possible function in extracellular proteolysis of thyroglobulin. J Cell Sci (2000) 1.13
Characterization of full-length cDNAs and the gene coding for the human GM2 activator protein. FEBS Lett (1991) 1.13
Variant of GM2-gangliosidosis with hexosaminidase A having a severely changed substrate specificity. EMBO J (1983) 1.13
[Resolution of mammalian N-acetyl-beta-D-hexosaminidase into multiple forms by electrofocussing]. Hoppe Seylers Z Physiol Chem (1968) 1.12
Structure of full-length cDNA coding for sulfatide activator, a Co-beta-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator. J Biochem (1989) 1.12
In vivo iodination of a misfolded proinsulin reveals co-localized signals for Bip binding and for degradation in the ER. EMBO J (1995) 1.12
Sulfatide activator protein. Alternative splicing that generates three mRNAs and a newly found mutation responsible for a clinical disease. J Biol Chem (1991) 1.10
The precursor of sulfatide activator protein is processed to three different proteins. Biol Chem Hoppe Seyler (1988) 1.09
The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A. J Biol Chem (1991) 1.08
Deletion of arginine (608) in acid sphingomyelinase is the prevalent mutation among Niemann-Pick disease type B patients from northern Africa. Hum Genet (1993) 1.08
Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor. J Biol Chem (1996) 1.07
Pathways of endocytosis from luminal plasma membrane in rat exocrine pancreas. Eur J Cell Biol (1980) 1.07
Migration of epidermal keratinocytes: mechanisms, regulation, and biological significance. Protoplasma (2004) 1.06
Uncoupling of ganglioside biosynthesis by Brefeldin A. Eur J Cell Biol (1990) 1.06
Human acid ceramidase: processing, glycosylation, and lysosomal targeting. J Biol Chem (2001) 1.06
Molecular genetics of GM2-gangliosidosis AB variant: a novel mutation and expression in BHK cells. Hum Genet (1993) 1.05
Modulation of ganglioside biosynthesis in primary cultured neurons. J Neurochem (1989) 1.05
Over-expression of a functionally active human GM2-activator protein in Escherichia coli. Biochem J (1993) 1.04
Endogenous peroxidase in the lacrimal gland of the rat and its differentiation against injected catalase and horseradish-peroxidase. Histochemie (1972) 1.04