Published in J Biol Chem on March 16, 2001
The major sites of cellular phospholipid synthesis and molecular determinants of Fatty Acid and lipid head group specificity. Mol Biol Cell (2002) 1.63
Phosphatidylcholine and the CDP-choline cycle. Biochim Biophys Acta (2012) 1.28
Phosphatidylcholine biosynthesis during neuronal differentiation and its role in cell fate determination. J Biol Chem (2010) 0.86
Delayed lysosomal metabolism of lipids in mucolipidosis type IV fibroblasts after LDL-receptor-mediated endocytosis. J Inherit Metab Dis (2001) 0.84
A presenilin-1-dependent gamma-secretase-like protease mediates release of Notch intracellular domain. Nature (1999) 10.36
Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein. Nature (1998) 7.43
Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice. Nature (2000) 6.34
mu1A-adaptin-deficient mice: lethality, loss of AP-1 binding and rerouting of mannose 6-phosphate receptors. EMBO J (2000) 4.06
Suppression and recovery of adrenal response after short-term, high-dose glucocorticoid treatment. Lancet (2000) 3.96
The frequency of lysosomal storage diseases in The Netherlands. Hum Genet (1999) 3.54
Presenilin 2 deficiency causes a mild pulmonary phenotype and no changes in amyloid precursor protein processing but enhances the embryonic lethal phenotype of presenilin 1 deficiency. Proc Natl Acad Sci U S A (1999) 2.82
Cathepsins B and D are dispensable for major histocompatibility complex class II-mediated antigen presentation. Proc Natl Acad Sci U S A (1998) 2.75
Destabilization of beta-catenin by mutations in presenilin-1 potentiates neuronal apoptosis. Nature (1998) 2.56
Sanfilippo syndrome: a mini-review. J Inherit Metab Dis (2008) 2.35
Dimensionality control of electronic phase transitions in nickel-oxide superlattices. Science (2011) 2.34
Direct measurement of the electronic spin diffusion length in a fully functional organic spin valve by low-energy muon spin rotation. Nat Mater (2008) 2.33
Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons. J Neurosci (2000) 2.21
Cathepsin L deficiency as molecular defect of furless: hyperproliferation of keratinocytes and pertubation of hair follicle cycling. FASEB J (2000) 2.09
Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency. Am J Hum Genet (1999) 1.99
Quantitative determination of phospholipid compositions by ESI-MS: effects of acyl chain length, unsaturation, and lipid concentration on instrument response. J Lipid Res (2001) 1.92
Dimethylmethylene blue-based spectrophotometry of glycosaminoglycans in untreated urine: a rapid screening procedure for mucopolysaccharidoses. Clin Chem (1989) 1.91
Cell surface presenilin-1 participates in the gamma-secretase-like proteolysis of Notch. J Biol Chem (1999) 1.82
Lateral organization of liquid-crystalline cholesterol-dimyristoylphosphatidylcholine bilayers. Evidence for domains with hexagonal and centered rectangular cholesterol superlattices. Biochemistry (1995) 1.80
The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein. J Biol Chem (2001) 1.76
Dissecting the role of the golgi complex and lipid rafts in biosynthetic transport of cholesterol to the cell surface. Proc Natl Acad Sci U S A (2000) 1.67
Disruption of the cathepsin K gene reduces atherosclerosis progression and induces plaque fibrosis but accelerates macrophage foam cell formation. Circulation (2005) 1.66
Plasma globotriaosylsphingosine: diagnostic value and relation to clinical manifestations of Fabry disease. Biochim Biophys Acta (2010) 1.65
Early embryonic death of mice deficient in gamma-adaptin. J Biol Chem (1999) 1.64
Donor-derived cells in the central nervous system of twitcher mice after bone marrow transplantation. Science (1988) 1.60
A fluorimetric enzyme assay for the diagnosis of Morquio disease type A (MPS IV A). Clin Chim Acta (1990) 1.57
Cathepsin D targeted by acid sphingomyelinase-derived ceramide. EMBO J (1999) 1.56
An improved two dimensional thin-layer chromatography system for the separation of phosphatidylglycerol and its derivatives. J Lipid Res (1976) 1.54
Proteases involved in MHC class II antigen presentation. Immunol Rev (1999) 1.54
Phospholipase A2 is present in meconium and inhibits the activity of pulmonary surfactant: an in vitro study. Acta Paediatr (2001) 1.50
The discrepancy between presenilin subcellular localization and gamma-secretase processing of amyloid precursor protein. J Cell Biol (2001) 1.46
ADAM10 regulates FasL cell surface expression and modulates FasL-induced cytotoxicity and activation-induced cell death. Cell Death Differ (2007) 1.46
Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy. Proc Natl Acad Sci U S A (1996) 1.44
The bone lining cell: its role in cleaning Howship's lacunae and initiating bone formation. J Bone Miner Res (2002) 1.41
Normal lysosomal morphology and function in LAMP-1-deficient mice. J Biol Chem (1999) 1.41
Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins. EMBO J (1993) 1.31
Elevated plasma chitotriosidase activity in various lysosomal storage disorders. J Inherit Metab Dis (1995) 1.28
The total free radical trapping ability of cord blood plasma in preterm and term babies. Pediatr Res (1989) 1.23
Potential role for cathepsin D in p53-dependent tumor suppression and chemosensitivity. Oncogene (1998) 1.22
Bone density, strength, and formation in adult cathepsin K (-/-) mice. Bone (2008) 1.19
The mechanism of human plasma phospholipid transfer protein-induced enlargement of high-density lipoprotein particles: evidence for particle fusion. Biochem J (1996) 1.19
Overlapping functions of lysosomal acid phosphatase (LAP) and tartrate-resistant acid phosphatase (Acp5) revealed by doubly deficient mice. Development (2001) 1.19
Clinical and genetic spectrum of Sanfilippo type C (MPS IIIC) disease in The Netherlands. Mol Genet Metab (2007) 1.18
Sanfilippo type B syndrome (mucopolysaccharidosis III B): allelic heterogeneity corresponds to the wide spectrum of clinical phenotypes. Eur J Hum Genet (1999) 1.17
Functions of cathepsin K in bone resorption. Lessons from cathepsin K deficient mice. Adv Exp Med Biol (2000) 1.17
Mass spectrometric analysis reveals an increase in plasma membrane polyunsaturated phospholipid species upon cellular cholesterol loading. Biochemistry (2001) 1.16
Phospholipid composition of the mammalian red cell membrane can be rationalized by a superlattice model. Proc Natl Acad Sci U S A (1998) 1.16
Depletion of mitochondrial DNA in the liver of a patient with lactic acidemia and hypoketotic hypoglycemia. J Pediatr (1996) 1.13
Membrane binding mechanism of an RNA virus-capping enzyme. J Biol Chem (2000) 1.11
Identification of 31 novel mutations in the N-acetylgalactosamine-6-sulfatase gene reveals excessive allelic heterogeneity among patients with Morquio A syndrome. Hum Mutat (1997) 1.10
Involvement of nitric oxide released from microglia-macrophages in pathological changes of cathepsin D-deficient mice. J Neurosci (2001) 1.10
Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI. Proc Natl Acad Sci U S A (1996) 1.10
Implication of APP secretases in notch signaling. J Mol Neurosci (2001) 1.09
Ceramide as an activator lipid of cathepsin D. Adv Exp Med Biol (2000) 1.08
Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis. Hum Mol Genet (1999) 1.07
Dicarboxylicaciduria and secondary carnitine deficiency in glycogenosis type IV. Arch Dis Child (1987) 1.07
Spermatogenesis proceeds normally in mice without linker histone H1t. Histochem Cell Biol (2000) 1.06
Cathepsin D is up-regulated in inflammatory bowel disease macrophages. Clin Exp Immunol (2004) 1.05
Presenilin-1 deficiency leads to loss of Cajal-Retzius neurons and cortical dysplasia similar to human type 2 lissencephaly. Curr Biol (1999) 1.04
Spatially homogeneous ferromagnetism of (Ga, Mn)As. Nat Mater (2010) 1.04
Engineering spin propagation across a hybrid organic/inorganic interface using a polar layer. Nat Mater (2010) 1.02
Transversal distribution of acyl-linked pyrene moieties in liquid-crystalline phosphatidylcholine bilayers. A fluorescence quenching study. Biochemistry (1995) 1.02
Glucocerebrosidase genotype of Gaucher patients in The Netherlands: limitations in prognostic value. Hum Mutat (1997) 1.02
Reduced sperm count and normal fertility in male mice with targeted disruption of the ADP-ribosylation factor-like 4 (Arl4) gene. Mol Cell Biol (2002) 1.02
gamma-cyclodextrins greatly enhance translocation of hydrophobic fluorescent phospholipids from vesicles to cells in culture. Importance of molecular hydrophobicity in phospholipid trafficking studies. J Biol Chem (1999) 1.02
Evidence for superlattice arrangements in fluid phosphatidylcholine/phosphatidylethanolamine bilayers. Biophys J (1997) 1.01
Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset. Ann Neurol (1998) 1.01
Effect of bone marrow transplantation on enzyme levels and clinical course in the neurologically affected twitcher mouse. J Clin Invest (1988) 0.99
Human alpha-N-acetylgalactosaminidase (alpha-NAGA) deficiency: no association with neuroaxonal dystrophy? Eur J Hum Genet (2001) 0.99
Improved estimation of cholesteryl ester transfer/exchange activity in serum or plasma. Clin Chem (1986) 0.99
Dipyrenylphosphatidylcholines as membrane fluidity probes. Relationship between intramolecular and intermolecular excimer formation rates. Biophys J (1990) 0.98
Cathepsin K deficiency in pycnodysostosis results in accumulation of non-digested phagocytosed collagen in fibroblasts. Calcif Tissue Int (2003) 0.97
Echinacea/sage or chlorhexidine/lidocaine for treating acute sore throats: a randomized double-blind trial. Eur J Med Res (2009) 0.97
Fluorescence studies of dehydroergosterol in phosphatidylethanolamine/phosphatidylcholine bilayers. Biophys J (1999) 0.96
Dipyrenylphosphatidylcholines as membrane fluidity probes. Pressure and temperature dependence of the intramolecular excimer formation rate. Biophys J (1993) 0.95
The effect of fasting and refeeding on the composition and synthesis of triacylglycerols, phosphatidylcholines, and phosphatidylethanolamines in rat liver. Arch Biochem Biophys (1979) 0.95
The twitcher mouse. Central nervous system pathology after bone marrow transplantation. Lab Invest (1988) 0.95
Acyl chain-dependent effect of lysophosphatidylcholine on human neutrophils. J Leukoc Biol (2007) 0.95
Bioavailability and pharmacokinetics of Echinacea purpurea preparations and their interaction with the immune system. Int J Clin Pharmacol Ther (2006) 0.95
Coexistence and coupling of superconductivity and magnetism in thin film structures. Phys Rev Lett (2005) 0.94
Transbilayer distribution and mobility of phosphatidylcholine in intact erythrocyte membranes. A study with phosphatidylcholine exchange protein. Eur J Biochem (1980) 0.93
Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disorders. Clin Chem (1995) 0.93
Influence of glucagon on the synthesis of phosphatidylcholines and phosphatidylethanolamines in monolayer cultures of rat hepatocytes. FEBS Lett (1979) 0.93
Elevation of beta-amyloid peptide 2-42 in sporadic and familial Alzheimer's disease and its generation in PS1 knockout cells. J Biol Chem (2001) 0.92
Spermatogenesis in mice is not affected by histone H1.1 deficiency. Exp Cell Res (2000) 0.92
Short-term manipulation of plasma free fatty acids does not change skeletal muscle concentrations of ceramide and glucosylceramide in lean and overweight subjects. J Clin Endocrinol Metab (2007) 0.92
Nanoscale layering of antiferromagnetic and superconducting phases in Rb(2)Fe(4)Se(5) single crystals. Phys Rev Lett (2012) 0.91
Intramolecular excimer formation of pyrene-labeled lipids in lamellar and inverted hexagonal phases of lipid mixtures containing unsaturated phosphatidylethanolamine. Biophys Chem (1991) 0.91
Different locations of cholesteryl ester transfer protein and phospholipid transfer protein activities in plasma. Atherosclerosis (1991) 0.90
Sleep improving effects of a single dose administration of a valerian/hops fluid extract - a double blind, randomized, placebo-controlled sleep-EEG study in a parallel design using electrohypnograms. Eur J Med Res (2008) 0.89
Cholesteryl ester transfer activity. Localization and role in distribution of cholesteryl ester among lipoproteins in man. Atherosclerosis (1984) 0.89
A sphingomyelin-transferring protein from chicken liver. Use of pyrene-labeled phospholipid. J Biol Chem (1995) 0.89
Metabolism of pyrenyl fatty acids in baby hamster kidney fibroblasts. Effect of the acyl chain length. J Biol Chem (1992) 0.88
Redundancy and specificity of the metalloprotease system mediating oncogenic NOTCH1 activation in T-ALL. Leukemia (2011) 0.88
Correction of lysosomal enzyme deficiency in various organs of beta-glucuronidase-deficient mice by allogeneic bone marrow transplantation. Transplantation (1987) 0.88
Safety and Efficacy Profile of Echinacea purpurea to Prevent Common Cold Episodes: A Randomized, Double-Blind, Placebo-Controlled Trial. Evid Based Complement Alternat Med (2012) 0.88
Effect of fasting and feeding a high-sucrose, fat-free diet on the synthesis of hepatic glycerolipids in vivo and in isolated hepatocytes. Biochim Biophys Acta (1977) 0.88
Cytochrome P450 inhibitory action of Echinacea preparations differs widely and co-varies with alkylamide content. J Pharm Pharmacol (2007) 0.88
What patients want to know before they have cataract surgery. Br J Ophthalmol (2004) 0.88