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A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)

American journal of human genetics, 1988-03, Vol.42 (3), p.422-434

1988

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Human Peroxisomal 3-oxoacyl-coenzyme A Thiolase Deficiency

Proceedings of the National Academy of Sciences - PNAS, 1987-04, Vol.84 (8), p.2494-2496

1987

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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

Biochemical and biophysical research communications, 1988-06, Vol.153 (2), p.618-624

1988

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Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions: a study using complementation analysis

The Journal of clinical investigation, 1988-06, Vol.81 (6), p.1710-1715

1988

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Biosynthesis and Maturation of Peroxisomal β -oxidation Enzymes in Fibroblasts in Relation to the Zellweger Syndrome and Infantile Refsum Disease

Proceedings of the National Academy of Sciences - PNAS, 1986-08, Vol.83 (16), p.6156-6158

1986

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Peroxisomal fatty acid β-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

The Journal of clinical investigation, 1987-12, Vol.80 (6), p.1778-1783

1987

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Kinetics of the assembly of peroxisomes after fusion of complementary cell lines from patients with the cerebro-hepato-renal (Zellweger) syndrome and related disorders

Biochemical and biophysical research communications, 1988-05, Vol.152 (3), p.1083-1089

1988

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X-Linked adrenoleukodystrophy: Defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters

Clinica chimica acta, 1987-06, Vol.165 (2), p.321-329

1987

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Peroxisomal β-oxidation enzyme proteins in the Zellweger syndrome

Biochemical and biophysical research communications, 1985-02, Vol.126 (3), p.1269-1275

1985

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Glucocerebrosidase, a lysosomal enzyme that does not undergo oligosaccharide phosphorylation

Biochimica et biophysica acta, 1988-03, Vol.964 (3), p.303-308

1988

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Peroxisomal very long-chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders

Clinica chimica acta, 1987-07, Vol.166 (2), p.255-263

1987

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Infantile Refsum disease: an inherited peroxisomal disorder: comparison with Zellweger syndrome and neonatal adrenoleukodystrophy

European journal of pediatrics, 1987-09, Vol.146 (5), p.477-483

1987

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Acid sphingomyelinase from human urine: purification and characterization

Biochimica et biophysica acta, 1987-12, Vol.922 (3), p.323-336

1987

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A procedure for the rapid purification in high yield of human glucocerebrosidase using immunoaffinity chromatography with monoclonal antibodies

Analytical biochemistry, 1986-05, Vol.154 (2), p.655-663

1986

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Bile acid analyses in “pseudo‐Zellweger” syndrome; clues to the defect in peroxisomal β‐oxidation

Journal of inherited metabolic disease, 1988-01, Vol.11 (S2), p.165-168

1988

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Efficient routing of glucocerebrosidase to lysosomes requires complex oligosaccharide chain formation

Biochemical and biophysical research communications, 1986-12, Vol.141 (2), p.452-458

1986

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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

Journal of inherited metabolic disease, 1988-06, Vol.11 (S2), p.173-177

1988

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Comparative study on glucocerebrosidase in spleens from patients with Gaucher disease

Biochemical journal, 1990-07, Vol.269 (1), p.93-100

1990

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Characterization of glucocerebrosidase in peripheral blood cells and cultured blastoid cells

Experimental cell research, 1988-08, Vol.177 (2), p.391-398

1988

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Comparison of the properties of a soluble form of glucocerebrosidase from human urine with those of the membrane-associated tissue enzyme

Biochimica et biophysica acta, 1986-12, Vol.863 (1), p.63-70

1986

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Infantile Refsum disease: deficiency of catalase-containing particles (peroxisomes), alkyldihydroxyacetone phosphate synthase and peroxisomal β-oxidation enzyme proteins

European journal of pediatrics, 1986-08, Vol.145 (3), p.172-175

1986

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Heterogeneity in human acid β-glucosidase revealed by cellulose-acetate electrophoresis

Biochimica et biophysica acta, 1988-05, Vol.965 (2), p.163-168

1988

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Deficient activity of glucocerebrosidase in urine from patients with type 1 Gaucher disease

Clinica chimica acta, 1986-07, Vol.158 (2), p.155-163

1986

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The identification of type 1 Gaucher disease patients, asymptomatic cases and carriers in The Netherlands using urine samples: An evaluation

Clinica chimica acta, 1991-12, Vol.203 (2), p.349-361

1991

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Cytochrome c oxidase: organ-specific isoenzymes and deficiencies

Journal of inherited metabolic disease, 1988-01, Vol.11, p.202-204

1988

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A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)

Human Peroxisomal 3-oxoacyl-coenzyme A Thiolase Deficiency

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

Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions: a study using complementation analysis

Biosynthesis and Maturation of Peroxisomal β -oxidation Enzymes in Fibroblasts in Relation to the Zellweger Syndrome and Infantile Refsum Disease

Peroxisomal fatty acid β-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

Kinetics of the assembly of peroxisomes after fusion of complementary cell lines from patients with the cerebro-hepato-renal (Zellweger) syndrome and related disorders

X-Linked adrenoleukodystrophy: Defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters

Peroxisomal β-oxidation enzyme proteins in the Zellweger syndrome

Glucocerebrosidase, a lysosomal enzyme that does not undergo oligosaccharide phosphorylation

Peroxisomal very long-chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders

Infantile Refsum disease: an inherited peroxisomal disorder: comparison with Zellweger syndrome and neonatal adrenoleukodystrophy

Acid sphingomyelinase from human urine: purification and characterization

A procedure for the rapid purification in high yield of human glucocerebrosidase using immunoaffinity chromatography with monoclonal antibodies

Bile acid analyses in “pseudo‐Zellweger” syndrome; clues to the defect in peroxisomal β‐oxidation

Efficient routing of glucocerebrosidase to lysosomes requires complex oligosaccharide chain formation

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

Comparative study on glucocerebrosidase in spleens from patients with Gaucher disease

Characterization of glucocerebrosidase in peripheral blood cells and cultured blastoid cells

Comparison of the properties of a soluble form of glucocerebrosidase from human urine with those of the membrane-associated tissue enzyme

Infantile Refsum disease: deficiency of catalase-containing particles (peroxisomes), alkyldihydroxyacetone phosphate synthase and peroxisomal β-oxidation enzyme proteins

Heterogeneity in human acid β-glucosidase revealed by cellulose-acetate electrophoresis

Deficient activity of glucocerebrosidase in urine from patients with type 1 Gaucher disease

The identification of type 1 Gaucher disease patients, asymptomatic cases and carriers in The Netherlands using urine samples: An evaluation

Cytochrome c oxidase: organ-specific isoenzymes and deficiencies

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