PubRank

U N Wiesmann

Author PubWeight™ 42.70‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Multiple lysosomal deficiency due to enzyme leakage? N Engl J Med 1971 1.55
2 Studies on the pathogenetic mechanism of I-cell disease in cultured fibroblasts. Pediatr Res 1974 1.44
3 Multiple sulfatase deficiencies in cultured skin fibroblasts. Occurrence in patients with a variant form of metachromatic leukodystrophy. Arch Neurol 1974 1.15
4 Effect of chloroquine on cultured fibroblasts: release of lysosomal hydrolases and inhibition of their uptake. Biochem Biophys Res Commun 1975 1.15
5 Mutation of ornithine transcarbamylase (H136R) in a girl with severe intermittent orotic aciduria but normal enzyme activity. J Inherit Metab Dis 1997 1.08
6 Dissociated cultures of newborn mouse brain. I. Metabolism of sulfated lipids and mucopolysaccharides. Neurobiology 1975 0.95
7 Role of petasin in the potential anti-inflammatory activity of a plant extract of petasites hybridus. Biochem Pharmacol 2001 0.95
8 Systemic carnitine deficiency due to lack of electron transfer flavoprotein:ubiquinone oxidoreductase. Neurology 1986 0.92
9 Treatment with low-dose diazoxide in two growth-retarded prepubertal girls with glycogen storage disease type Ia resulted in catch-up growth. J Inherit Metab Dis 1997 0.91
10 Unusually mild course of beta-glucuronidase deficiency in two brothers (mucopolysaccharidosis VII). Helv Paediatr Acta 1978 0.91
11 Mucolipidosis II (I-cell disease). A clinical and biochemical study. Acta Paediatr Scand 1974 0.90
12 Antioxidant and thyroid hormone status in selenium-deficient phenylketonuric and hyperphenylalaninemic patients. Am J Clin Nutr 2000 0.87
13 Prenatal mucopolysaccharidosis II (Hunter): a pathogenetic study. Pediatr Res 1980 0.87
14 Correction of the defective sulfatide degradation in cultured fibroblasts from patients with metachromatic leucodystrophy. Acta Paediatr Scand 1972 0.87
15 Sodium concentration in unstimulated parotid saliva and on oral mucosa in normal subjects and in patients with cystic fibrosis. J Pediatr 1970 0.86
16 Membrane adsorption and internalization of (14C)chloroquine by cultured human fibroblasts. Biochem Pharmacol 1977 0.86
17 Flow-rates and electrolytes in minor-salivary-gland saliva in normal subjects and patients with cystic fibrosis. Lancet 1972 0.86
18 Vesicular transport of sulfatide in the myelinating mouse brain. Functional association with lysosomes? J Biol Chem 1982 0.85
19 Organ-specific, qualitative changes in the phospholipid composition of rats after chronic administration of the antidepressant drug desipramine. Biochem Pharmacol 1988 0.84
20 Ceramide-galactosyltransferase and cerebroside-sulphotranserase localisation in Golgi membranes isolated by a continuous sucrose gradient of mouse brain microsomes. J Neurochem 1979 0.84
21 Combined 3-methylglutaconic and 3-hydroxy-3-methylglutaric aciduria with endocardial fibroelastosis and dilatative cardiomyopathy in male and female siblings with partial deficiency of complex II/III in fibroblasts. Enzyme Protein 1996 0.83
22 Evidence for lysosomotropic action of desipramine in cultured human fibroblasts. J Pharmacol Exp Ther 1983 0.83
23 Effects of culture and incubation conditions on membrane fluidity in monolayers of cultured cells measured as fluorescence anisotropy using trimethylammoniumdiphenylhexatriene (TMA-DPH). Biochim Biophys Acta 1990 0.82
24 Vitamin E reduces accumulation of amiodarone and desethylamiodarone and inhibits phospholipidosis in cultured human cells. Biochem Pharmacol 1995 0.82
25 Regulatory adaptation of isoprenoid biosynthesis and the LDL receptor pathway in fibroblasts from patients with mevalonate kinase deficiency. Pediatr Res 1997 0.81
26 Niemann-Pick's disease. Clinical, biochemical and ultrastructural findings in a case of the infantile form. J Neurol 1975 0.81
27 Enhanced adenosine 3':5' - monophosphate response to beta-adrenergic stimulation in cystic fibrosis fibroblasts after removal of conditioned medium. Pediatr Res 1980 0.81
28 Evidence for lysosomotropism of memantine in cultured human cells: cellular kinetics and effects of memantine on phospholipid content and composition, membrane fluidity and beta-adrenergic transmission. Pharmacol Toxicol 1993 0.80
29 Prenatal metachromatic leukodystrophy. Helv Paediatr Acta 1975 0.80
30 Quantitative measurement of in vivo sulfatide metabolism during development of the mouse brain: evidence for a large rapidly degradable sulfatide pool. Dev Biol 1981 0.80
31 Generalized gangliosidosis: acid beta-galactosidase deficiency with early onset, rapid mental deterioration and minimal bone dysplasia. J Neurol 1976 0.79
32 Subcellular distribution of the antidepressant drug desipramine in cultured human fibroblasts after chronic administration. Drug-effect on the subcellular distribution of accumulated phospholipids. Biochem Pharmacol 1987 0.79
33 Changes in beta adrenergic receptors in submaxillary glands of chronically reserpine- or isoproterenol-treated rats. J Pharmacol Exp Ther 1981 0.79
34 Cellular accumulation of amiodarone and desethylamiodarone in cultured human cells. Consequences of drug accumulation on cellular lipid metabolism and plasma membrane properties of chronically exposed cells. Biochem Pharmacol 1993 0.78
35 Juxtacrine stimulation of cytokine production in cocultures of human dermal fibroblasts and T cells. Cytokine 1996 0.78
36 Induction of multinucleate cells by 8-MOP and UV treatment in vitro and in vivo. Dermatologica 1977 0.78
37 Chronic exposure of human cells in culture to the tricyclic antidepressant desipramine reduces the number of beta-adrenoceptors. Biochem Pharmacol 1986 0.78
38 Chondrodysplasia punctata with a mild clinical course. J Inherit Metab Dis 1994 0.78
39 Specific and non specific stimulation of prostaglandin release by human skin fibroblasts in culture.--Are changes of membrane fluidity involved? Prostaglandins 1992 0.77
40 Uptake and intracellular stability of glycosylphosphatidylinositol-specific phospholipase D in neuroblastoma cells. Biochim Biophys Acta 1997 0.77
41 Effects of bile and bile acids on cultured human fibroblasts. Eur J Clin Invest 1977 0.77
42 Chemically induced plasma membrane vesicles as a useful tool for the investigation of virus binding to susceptible cells. J Virol Methods 1993 0.77
43 Differential inhibition of inflammatory effector functions by petasin, isopetasin and neopetasin in human eosinophils. Clin Exp Allergy 2001 0.77
44 Autologous versus allogeneic T cell-stimulated IL-6 production by dermal fibroblasts. Inflammation 1997 0.76
45 Inhibition by vitamin E of drug accumulation and of phospholipidosis induced by desipramine and other cationic amphiphilic drugs in human cultured cells. Br J Pharmacol 1996 0.76
46 Acetylcholinesterase in mouse neuroblastoma NB2A cells: analysis of production, secretion, and molecular forms. J Neurochem 1989 0.76
47 Net sulfatide synthesis, galactosylceramide sulfotransferase and arylsulfatase A activity in the developing cerebrum and cerebellum of normal mice and myelin-deficient jimpy mice. Biochim Biophys Acta 1981 0.76
48 Cultured human skin fibroblasts modify their plasma membrane lipid composition and fluidity according to growth temperature suggesting homeoviscous adaptation at hypothermic (30 degrees C) but not at hyperthermic (40 degrees C) temperatures. Biochim Biophys Acta 1992 0.76
49 Hypericum perforatum L. (St John's wort) extract Ze 117 inhibits dopamine re-uptake in rat striatal brain slices. An implication for use in smoking cessation treatment? Phytother Res 2010 0.76
50 Multiple abnormalities of ectoglycosyltransferases in cultured fibroblasts from patients with mucolipidosis II: Possible indication for abnormal plasma membrane glycoproteins. Pediatr Res 1977 0.75
51 Insulin resistance and membrane abnormalities. Lancet 1995 0.75
52 3'-phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase. An optimized assay in homogenates of developing brain. Biochim Biophys Acta 1977 0.75
53 Differential regulation of bradykinin receptor density, intracellular Ca2+, and prostanoid release in skin and foreskin fibroblasts. Effects of cell density and interleukin-1alpha. Br J Pharmacol 1999 0.75
54 Phospholipid composition of cultivated skin fibroblasts in Duchenne's muscular dystrophy. Clin Chim Acta 1976 0.75
55 Ultrastructure of lipids in the optic nerve of the mouse mutant "jimpy". A study of histochemistry, freeze-etching, and thin section technique. Acta Neuropathol 1979 0.75
56 Sulfated glycosaminoglycans (GAG) in the developing mouse brain. Quantitative aspects on the metabolism of total and individual sulfated GAG in vivo. Dev Biol 1987 0.75
57 Natural killer cells activate human dermal fibroblasts. Cytokine 2000 0.75
58 Confirming by electron microscopy a prenatally diagnosed storage disorder: a note of caution. Helv Paediatr Acta 1975 0.75
59 An unusual form of arylsulfatase A deficiency combined with sulfatide-excretion and a normal sulfatide-loading. Acta Paediatr Scand 1983 0.75
60 [Lysosomal storage diseases: clinical variety and genetic heterogeneity (author's transl)]. Verh Dtsch Ges Pathol 1976 0.75
61 [Concepts of lysosomal storage disease--known facts and hypotheses]. Verh Dtsch Ges Pathol 1982 0.75
62 Metabolism of sulfogalactosyl glycerolipids in the myelinating mouse brain. Dev Biol 1983 0.75
63 Effects of antibiotics on the growth and differentiation in dissociated brain cell cultures. Neuroscience 1978 0.75
64 Atypical metachromatic leukodystrophy? Problems with the biochemical diagnosis. Hum Genet 1984 0.75
65 [Lysosomal storage diseases: possibilities of the ultrastructural diagnosis (author's transl)]. Verh Dtsch Ges Pathol 1976 0.75
66 Arylsulfatase A in developing normal and jimpy mice. Neuroscience 1977 0.75
67 Relevance of second messengers in cystic fibrosis. Monogr Paediatr 1979 0.75
68 Mild form of the Hunter syndrome, indentity of the biochemical defect with the severe type. Helv Paediatr Acta 1974 0.75
69 Abnormal metabolism of 35SO4-sulfatide in jimpy brains expressed in brain organotypic cultures. J Neuropathol Exp Neurol 1980 0.75
70 [Assessment of the nephrotoxicity of amikacin in patients with cystic fibrosis]. Schweiz Med Wochenschr 1992 0.75
71 A biochemical approach to lipid storage myopathies. Biochem Exp Biol 1977 0.75
72 Synthesis and subcellular transport of sulfogalactosyl glycerolipids in the myelinating mouse brain. Biochim Biophys Acta 1983 0.75
73 Single and multiple desipramine exposures of cultured cells. Changes in cellular anisotropy and in lipid composition of whole cells and of plasma membranes. Biochem Pharmacol 1990 0.75
74 Mucocutaneous bleeding, a rare but severe complication in nephrosialidosis. Eur J Pediatr 1994 0.75
75 Late infantile metachromatic leucodystrophy (MLD). Clinical and diagnostic evaluation in a typical case. J Neurol 1975 0.75
76 Treatment of metachromatic leukodystrophy in fibroblasts by enzyme replcement. N Engl J Med 1971 0.75
77 Weekly versus biweekly lipid removal and effect of statins in severe hypercholesterolemia. Am J Ther 1998 0.75
78 Elevated CSF cyclic AMP concentrations in patients with inflammatory diseases of cerebral and extracerebral origin. Eur J Pediatr 1980 0.75
79 Enzyme--cell membrane recognition (what we can learn from rare genetic lysosomal disorders). Biochem Exp Biol 1977 0.75
80 [Lysosomal storage diseases: possibilities of the clinical-biochemical diagnostics (author's transl)]. Verh Dtsch Ges Pathol 1976 0.75
81 Sialidoses (mucolipidoses), clinical pictures. Adv Exp Med Biol 1980 0.75
82 Medium-chain acyl-CoA dehydrogenase deficiency does not correlate with apparent life-threatening events and the sudden infant death syndrome: results from phenylpropionate loading tests and DNA analysis. Eur J Pediatr 1994 0.75
83 Soluble IL-1 receptor type I binds to human dermal fibroblasts and induces calcium flux. FEBS Lett 1998 0.75
84 Polyploidization and hemiploidization induced by PUVA in vivo. Dermatologica 1979 0.75
85 [Pathophysiology of sulfatide metabolism in metachromatic leukodystrophy]. Bull Schweiz Akad Med Wiss 1978 0.75