PubRank

C W van Roermund

Author PubWeight™ 39.90‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency. Proc Natl Acad Sci U S A 1987 1.98
2 The ABC transporter proteins Pat1 and Pat2 are required for import of long-chain fatty acids into peroxisomes of Saccharomyces cerevisiae. EMBO J 1996 1.72
3 Control of gluconeogenesis in rat liver cells. Flux control coefficients of the enzymes in the gluconeogenic pathway in the absence and presence of glucagon. Biochem J 1986 1.70
4 A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy). Am J Hum Genet 1988 1.59
5 Peroxisomal fatty acid alpha- and beta-oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases. Biochem Soc Trans 2001 1.52
6 Analysis of the carboxyl-terminal peroxisomal targeting signal 1 in a homologous context in Saccharomyces cerevisiae. J Biol Chem 1996 1.52
7 Peroxisomal and mitochondrial carnitine acetyltransferases of Saccharomyces cerevisiae are encoded by a single gene. EMBO J 1995 1.47
8 Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts. J Inherit Metab Dis 1995 1.31
9 Transport of activated fatty acids by the peroxisomal ATP-binding-cassette transporter Pxa2 in a semi-intact yeast cell system. Eur J Biochem 1997 1.19
10 Properties of carbamoyl-phosphate synthetase (ammonia) in rat-liver mitochondria made permeable with toluene. Eur J Biochem 1983 1.06
11 Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids. Biochem Biophys Res Commun 1988 1.04
12 Rhizomelic chondrodysplasia punctata. Deficiency of 3-oxoacyl-coenzyme A thiolase in peroxisomes and impaired processing of the enzyme. J Clin Invest 1990 1.01
13 Peroxisomal beta-oxidation of palmitoyl-CoA in human liver homogenates and its deficiency in the cerebro-hepato-renal (Zellweger) syndrome. Clin Chim Acta 1986 1.00
14 The inborn errors of peroxisomal beta-oxidation: a review. J Inherit Metab Dis 1990 1.00
15 Peroxisomal very long-chain fatty acid beta-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders. Clin Chim Acta 1987 0.98
16 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome. Biochim Biophys Acta 1994 0.94
17 Identification of pristanoyl-CoA oxidase activity in human liver and its deficiency in the Zellweger syndrome. Biochem Biophys Res Commun 1990 0.94
18 X-linked adrenoleukodystrophy: identification of the primary defect at the level of a deficient peroxisomal very long chain fatty acyl-CoA synthetase using a newly developed method for the isolation of peroxisomes from skin fibroblasts. J Inherit Metab Dis 1988 0.93
19 X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect. J Inherit Metab Dis 1992 0.92
20 Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders. J Clin Invest 1987 0.91
21 Alanine glyoxylate aminotransferase and the urinary excretion of oxalate and glycollate in hyperoxaluria type I and the Zellweger syndrome. Clin Chim Acta 1987 0.91
22 Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme protein. Biochem Biophys Res Commun 1997 0.86
23 A bifunctional protein with deficient enzymic activity: identification of a new peroxisomal disorder using novel methods to measure the peroxisomal beta-oxidation enzyme activities. J Inherit Metab Dis 1990 0.86
24 Di- and trihydroxycholestanaemia in twin sisters. J Inherit Metab Dis 1991 0.86
25 Functional analysis of mutant human carnitine acylcarnitine translocases in yeast. Biochem Biophys Res Commun 2001 0.85
26 Studies on the peroxisomal oxidation of palmitate and lignocerate in rat liver. Biochim Biophys Acta 1987 0.85
27 Bifunctional enzyme deficiency: identification of a new type of peroxisomal disorder in a patient with an impairment in peroxisomal beta-oxidation of unknown aetiology by means of complementation analysis. J Inherit Metab Dis 1992 0.84
28 X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters. Clin Chim Acta 1987 0.84
29 Prenatal diagnosis of Zellweger syndrome by measurement of very long chain fatty acid (C26:0) beta-oxidation in cultured chorionic villous fibroblasts: implications for early diagnosis of other peroxisomal disorders. Clin Chim Acta 1987 0.84
30 Identification of L-pipecolate oxidase in human liver and its deficiency in the Zellweger syndrome. Biochem Biophys Res Commun 1988 0.83
31 Metabolic aspects of peroxisomal disorders. Ann N Y Acad Sci 1996 0.82
32 Mechanism of the stimulation of respiration by fatty acids in rat liver. FEBS Lett 1985 0.79
33 Identification of pristanoyl-CoA oxidase and phytanic acid decarboxylation in peroxisomes and mitochondria from human liver: implications for Zellweger syndrome. J Inherit Metab Dis 1991 0.79
34 Catalase in cultured skin fibroblasts from patients with the cerebro-hepato-renal (Zellweger) syndrome: normal maturation in peroxisome-deficient cells. Biochim Biophys Acta 1987 0.79
35 Di- and trihydroxycholestanoic acidaemia with hepatic failure. J Inherit Metab Dis 1990 0.78
36 Subcellular fractionation of cultured normal human melanocytes: new insights into the relationship of melanosomes with lysosomes and peroxisomes. Biochim Biophys Acta 1993 0.77
37 Human liver L-alanine-glyoxylate aminotransferase: characteristics and activity in controls and hyperoxaluria type I patients using a simple spectrophotometric method. Clin Chim Acta 1990 0.77
38 Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome. J Inherit Metab Dis 1995 0.77
39 Subcellular localization of squalene synthase in human hepatoma cell line Hep G2. Biochim Biophys Acta 1992 0.77
40 Peroxisomes and peroxisomal functions in hyperpipecolic acidaemia. J Inherit Metab Dis 1988 0.77
41 Peroxisomes and peroxisomal functions in muscle. Studies with muscle cells from controls and a patient with the cerebro-hepato-renal (Zellweger) syndrome. Exp Cell Res 1987 0.76
42 Characteristics and subcellular localization of pristanoyl-CoA synthetase in rat liver. Biochim Biophys Acta 1992 0.76
43 Diversity in residual alanine glyoxylate aminotransferase activity in hyperoxaluria type I: correlation with pyridoxine responsiveness. J Inherit Metab Dis 1988 0.75
44 Regulation of mitochondrial respiration in liver. Adv Exp Med Biol 1986 0.75
45 Molecular analysis of disorders of peroxisomal beta-oxidation. Prog Clin Biol Res 1992 0.75
46 Impaired ability of peroxisomes to activate very-long-chain fatty acids in X-linked adrenoleukodystrophy. Lancet 1988 0.75
47 Application of control analysis in studies of regulation of intermediary metabolism. Biomed Biochim Acta 1985 0.75
48 Peroxisomal enzyme activities in the human hepatoblastoma cell line HepG2 as compared to human liver. Biochim Biophys Acta 1991 0.75
49 Accumulation and impaired in vivo metabolism of di- and trihydroxycholestanoic acid in two patients. Clin Chim Acta 1991 0.75
50 Inborn errors of peroxisomal beta-oxidation: biochemical identification using enzymic and immunological methods. Prog Clin Biol Res 1990 0.75