Summary
Peroxisomes play an essential role in human cellular metabolism. Peroxisomal disorders, a group of genetic diseases caused by peroxisomal dysfunction, can be classified into three groups: (1) disorders of peroxisome biogenesis with a generalized loss of peroxisomal functions (Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease, hyperpipecolic acidaemia); (2) disorders with a loss of multiple peroxisomal functions (rhizomelic chondrodysplasia punctata and Zellweger-like syndrome; (3) disorders with loss of a single peroxisomal function (X-linked adrenoleukodystrophy, peroxisomal thiolase deficiency, bifunctional protein deficiency, acyl-CoA oxidase deficiency, classic Refsum disease, hyperoxaluria type I and acatalasaemia). Prenatal diagnosis is indicated in all these genetic disorders with the exception of classic Refsum disease, most types of hyperoxaluria type I and acatalasaemia.
A variety of techniques is available now for the prenatal diagnosis of peroxisomal disorders in the first or second trimester of gestation. Prenatal diagnosis was performed by us in 70 pregnancies at risk for a disorder of peroxisome biogenesis, three for rhizomelic chondrodysplasia punctata, four for X-linked adrenoleukodystrophy and two for a defect in peroxisomal β-oxidation. Fourteen affected fetuses were identified; no false negative cases were obtained.
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References
Appelkvist, E. L. and Dallner, G. Dolichol metabolism and peroxisomes. In: Fahimi, H. D. and Sies, H. (Eds.),Peroxisomes in Biology and Medicine, Springer-Verlag, Berlin, 1987, pp. 53–66
Aubourg, P. R., Sack, G. H., Meyers, D. A., Lease, J. J. and Moser, H. W. Linkage of adrenoleukodystrophy to a polymorphic DNA probe.Ann. Neurol. 21 (1987) 349–352
Barth, P. G., Schutgens, R. B. H., Bakkeren, J. A. J. M., Dingemans, K. P., Heymans, H. S. A., Douwes, A. C. and van der Kley-van Moorsel, J. J. A milder variant of Zellweger syndrome.Eur. J. Pediatr. 144 (1985) 338–342
Beard. M. E., Baker, R., Conomos, P., Pugatch, D. and Holzman, E. Oxidation of oxalate and polyamines by rat liver peroxisomes.J. Histochem. Cytochem. 33 (1985) 460–464
Björkhem, I., Blomstrand, S., Glaumann, H. and Strandvik, B. Unsuccessful attempts to induce peroxisomes in two cases of Zellweger disease by treatment with clofibrate.Pediatr. Res. 19 (1985) 590–593
Bleeker-Wagemakers, E. M., Oorthuys, J. W. E., Wanders, R. J. A. and Schutgens, R. B. H. Long term survival of a patient with the cerebro-hepato-renal (Zellweger) syndrome.Clin. Genet. 29 (1986) 160–164
Boué, J., Oberle, I., Heilig, R., Mandel, J. L., Moser, H. W., Larsen, J. W. Jr., Dumez, Y. and Boué, A. First trimester prenatal diagnosis of adrenoleukodystrophy by determination of very long chain fatty acid levels and by linkage analysis to a DNA probe.Hum. Genet. 69 (1985) 272–274
Brul, S., Westerveld, A., Strijland, A., Wanders, R. J. A., Schram, A. W., Heymans, H. S. A., Schutgens, R. B. H., van den Bosch, H. and Tager, J. M. Genetic heterogeneity in the cerebro-hepato-renal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions: a study using complementation analysis.J. Clin. Invest. 81 (1988) 1710–1715
Carey, W. F., Robertson, E. F., van Crugten, C., Poulos, A. and Nelson, P. N. Prenatal diagnosis of Zellweger's syndrome by chorionic villus sampling — and a caveat.Prenat. Diagn. 6 (1986) 227–229
Danpure, C. J., and Jennings, P. R. Peroxisomal alanine: glyoxylate aminotransferase deficiency in primary hyperoxaluria type I.FEBS Lett., 201 (1986) 20–24
Danpure, C. J., Cooper, P. J., Jennings, P. R., Wise P. J., Penketh, R. J. and Rodeck, C. H. Enzymatic prenatal diagnosis of primary hyperoxaluria type 1: successes and potential problems.26th SSIEM Annual Symposium, 1988, Abstract 012
de Duve, C. and Baudhuin, P. Peroxisomes (microbodies and related particles).Physiol. Rev., 46 (1966) 323–357
Diczfalusy, U., Alexson, S. E. H. and Pedersen, J. I. Chain shortening of prostaglandin PGF2α.Biochem. Biophys. Res. Commun. 144 (1987) 1206–1213
Goldfischer, S., Moore, C. L., Johnson, A. B., Spiro, A. J., Valsamis, M. P., Wisniewski, H. K., Ritch, R. H., Norton, W. T., Rapin, I. and Gartner, L. M. Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome,Science 182 (1973) 62–64
Goldfischer, S. and Reddy, J. K. Peroxisomes (microbodies) in cell pathology.Int. Rev. Exp. Pathol. 26 (1984) 45–84
Goldfischer, S., Collins, J., Rapin, I., Coltoff-Schiller, B., Chang, C. H., Nigro, M., Black, V. H., Javitt, N. B., Moser, H. W. and Lazarow, P. B. Peroxisomal defects in neonatalonset and X-linked adrenoleukodystrophies.Science 227 (1985) 69–71
Goldfischer, S., Collins, J., Rapin, I., Neumann, P., Neglia, W., Spiro, A. J., Ishii, T., Roels, F., Vamecq, J. and van Hoof, F. Pseudo-Zellweger syndrome: deficiencies in several peroxisomal oxidative activities.J. Pediatr. 108 (1986) 25–32
Hajra, A. K. Biosynthesis of O-alkylglycerol ether lipids. In: Mangold, J. K. and Paltauf, F. (Eds.),Ether Lipids: Biochemical and biomedical aspects, Academic Press, Orlando, 1984, 85–106
Hajra, A. K., Datta, N. S., Jackson, L. B., Moser, A. B., Moser, H. W., Larsen, J. W. Jr. and Powers, J. Prenatal diagnosis of Zellweger cerebro-hepato-renal syndrome.N. Engl. J. Med. 312 (1985) 445–446
Heymans, H. S. A., Oorthuys, J. W. E., Nelck, G., Wanders, R. J. A., Dingemans, K. P. and Schutgens, R. B. H. Peroxisomal abnormalities in rhizomelic chondrodysplasia punctata.J. Inher. Metab. Dis. 9 Suppl. 2 (1986) 329–331
Hiltunen, J. K., Karki, T., Hassinen, I. E. and Osmundsen, H. β-oxidation of polyunsaturated fatty acids by rat liver peroxisomes. A role for 2,4-dienoyl-Coenzyme A reductase in peroxisomal β-oxidation.J. Biol. Chem. 261 (1986) 16484–16493
Hoefler, G., Hoefler, S., Watkins, P. A., Chen, W. W., Moser, A., Baldwin, V., McGillivary, B., Charrow, J., Friedman, J. M., Rutledge, L., Hashimoto, T. and Moser, H. W. Biochemical abnormalities in rhizomelic chondrodysplasia punctata.J. Pediatr. 112 (1988) 726–733
Höltta, E. Oxidation of spermine and spermidine in rat liver: purification and properties of polyamine oxidase.Biochemistry 16 (1977) 91–100
Hovik, R. and Osmundsen, H. Peroxisomal β-oxidation of long-chain fatty acids possessing different extents of unsaturation.Biochem. J. 247 (1987) 531–535
Kelley, R. I. The cerebro-hepato-renal syndrome of Zellweger. Morphological and metabolic aspect.Am. J. Med. Genet. 16 (1983) 502–517
Kolvraa, S. and Gregersen, N.In vitro studies on the oxidation of medium-chain dicarboxylic acids in rat liver.Biochim. Biophys. Acta 876 (1986) 515–525
Lazarow, P. B. Biogenesis of peroxisomes: Implications for Zellweger syndrome. In Vogel, F., Sperling, K. (Eds.),Human Genetics, Springer Verlag, Berlin, 1987, 369–376
Lazarow, P. B. and de Duve, C. A fatty acyl-CoA oxidizing system in rat liver peroxisomes: enhancement by clofibrate, a hypolipidemic drug.Proc. Natl. Acad. Sci. USA, 73 (1976) 2043–2046
Lazarow, P. B. and Moser, H. W. Disorders of Peroxisome biogenesis. In Scriver, C. R., Beaudet, A. L., Sly, W. S., Valle, D. (Eds.),The Metabolic Basis of Inherited Disease, 6th edn., McGraw-Hill, New York, 1989, in press
Lazarow, P. B., Black, V., Shio, H., Fujiki, Y., Hajra, A. K., Datta, N. S., Bangaru, B. S. and Dancis, J. Zellweger syndrome: biochemical and morphological studies on two patients treated with clofibrate.Pediatr. Res. 19 (1985) 1356–1364
Mihalik, S. J. and Rhead, W. J.l-pipecolic acid catabolism in mammals.Trans. Am. Soc. Neurochem. 19 (1988) 72 (Abstract)
Moser, H. W., Moser, A. B., Frayer, K. K., Chen, W., Schulman, J. D., O'Neill, B. P. and Kishimoto, Y. Adrenoleukodystrophy: Increased plasma content of saturated very long chain fatty acids.Neurology 31 (1981) 1241–1249
Moser, A. E., Singh, I., Brown, F. R. III, Solish, G. I., Kelley, R. I., Benke, P. J. and Moser, H. W. The cerebro-hepato-renal (Zellweger) syndrome: increased levels and impaired degradation of very long fatty acids and prenatal diagnosis.N. Engl. J. Med. 310 (1984) 1141–1146
Moser, H. W., Naidu, S., Kumar, A. J. and Rosenbaum, A. E. The adrenoleukodystrophies.Crit. Rev. Neurobiol., 5 (1987b) 29–88
Moser, A. E., Borel, J., Odone, A., Naidu, S., Cornblath, D., Sanders, D. B. and Moser, H. W. A new dietary therapy for adrenoleukodystrophy: biochemical and preliminary clinical results in 36 patients.Ann. Neurol. 21 (1987a) 240–249
Noguchi, T. Amino acid metabolism in animal peroxisomes. In Fahimi, H. D. and Sies, H. (Eds.),Peroxisomes in Biology and Medicine, Springer-Verlag, Berlin, 1987, 234–243
Novikoff, A. B., Novikoff, P. M., Davis, C. and Quintana, N. Studies on microperoxisomes. V. Are microperoxisomes ubiquitous in mammalian cells?J. Histochem. Cytochem. 21 (1973) 737–755
Paturneau-Jouas, E., Taillard, A., Gansmuller, J., Mikol, J. Aigrot, M. S. and Sereni, C. Clinical, biochemical and pathological aspects of a ‘Zellweger-like’ peroxisomal disorder. In Salvayre, R. (Ed.),Lipid Storage Disorders, Nato-Inserm, Toulouse (1987) 133–134 (Abstract)
Pedersen, J. J., Kase, B. F., Prydz, K. and Björkhem, I. Liver peroxisomes and bile acid formation. In Fahimi, H. D. and Sies, H. (Eds.),Peroxisomes in Biology and Medicine, Springer-Verlag, Berlin, 1987, 67–77
Poll-Thé, B. T., Poulos, A., Sharp, P., Boué, J., Ogier, H., Odievre, M. and Saudubray, J. M. Antenatal diagnosis of infantile Refsum's disease.Clin. Genet., 27 (1985) 524–526
Poll-Thé, B. T., Skjeldal, O. H., Stokke, O., Poulos, A., Demaugre, F. and Saudubray, J. M. Phytanic acid alpha-oxidation and complementation analysis of classical Refsum and peroxisomal disorders.Hum. Genet. 81 (1989) 175–181
Poll-Thé, B. T., Roels, F., Ogier, H., Scotto, J., Vamecq, J., Schutgens, R. B. H., van Roermund, C. W. T., van Wijland, M. J. A., Schram, A. W., Tager, J. M. and Saudubray, J. M. A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo neonatal adrenoleukodystrophy).Am. J. Hum. Genet. 42 (1988) 422–434
Poulos, A., Sharp, P. and Whiting, M. Infantile Refsum's disease (phytanic acid storage disease): a variant of Zellweger's syndrome?Clin. Genet. 25 (1984) 579–586
Poulos, A., van Crugten, C., Sharp, P., Carey, W. F., Robertson, E., Becroft, B. M. D., Saudubray, J. M., Poll-Thé, B. T., Christensen, E. and Brandt, N. Prenatal diagnosis of Zellweger syndrome and related disorders: impaired degradation of phytanic acid.Eur. J. Pediatr. 145 (1986) 507–510
Rizzo, W. B. Personal communication
Rizzo, W. B., Watkins, P. A., Phillips, M. W., Cranin, D., Campbell, B. and Avigan, J. Adrenoleukodystrophy: Oleic acid lowers fibroblast saturated C22–C26 fatty acids.Neurology 26 (1986) 357–361
Rocchiccioli, F., Aubourg, P. and Choiset, A. Immediate prenatal diagnosis of Zellweger syndrome by direct measurement of very long chain fatty acids in chorionic villus cells.Prenat. Diagn. 7 (1987) 349–354
Roels, F., Verdonck, V., Pauwels, M., Lissens, W. and Liebaers, I. Visualization of peroxisomes and plasmalogens in first trimester chorionic villus.J. Inher. Metab. Dis. 10 Suppl. 2 (1987) 349–354
Roscher, A., Hoefler, S., Hoefler, G., Paschke, E. and Paltauf, F. Neonatal adrenoleukodystrophy and cerebro-hepato-renal syndrome: genetic complementation analysis of impaired peroxisomal plasmalogen biosynthesis. InInborn Errors of Cellular Organelles, 24th SSIEM Symposium, Amersfoort, 1986, 03 (Abstract)
Schepers, L., Casteels, M., Vamecq, J., Parmentier, G., van Veldhoven, P. P. and Mannaerts, G. P. β-Oxidation of the carboxyl side chain of prostaglandin E2 in rat liver peroxisomes and mitochondria.J. Biol. Chem. 263 (1988) 2724–2731
Schrakamp, G., Schalkwijk, C. G., Schutgens, R. B. H., Wanders, R. J. A., Tager, J. M. and van den Bosch, H. Plasmalogen biosynthesis in peroxisomal disorders.J. Lipid Res. 29 (1988) 325–334
Schram, A. W., Strijland, A., Hashimoto, T., Wanders, R. J. A., Schutgens, R. B. H., van den Bosch, H. and Tager, J. M. Biosynthesis and maturation of peroxisomal β-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum disease.Proc. Natl. Acad. Sci. USA 83 (1986) 6156–6158
Schram, A. W., Goldfischer, S., van Roermund, C. W. T., Brouwer-Kelder, E. M., Collins, J., Hashimoto, T., Heymans, H. S. A., van den Bosch, H., Schutgens, R. B. H., Tager, J. M. and Wanders, R. J. A. Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.Proc. Natl. Acad. Sci. USA 84 (1987) 2494–2496
Schutgens, R. B. H., Schrakamp, G., Wanders, R. J. A., Heymans, H. S. A., Moser, H. W., Moser, A. E., Tager, J. M., van den Bosch, H. and Aubourg, P. The cerebrohepato-renal (Zellweger) syndrome: prenatal detection based on impaired biosynthesis of plasmalogens.Prenat. Diagn. 5 (1985) 337–344
Schutgens, R. B. H., Heymans, H. S. A., Wanders, R. J. A., van den Bosch, H. and Tager, J. M. Peroxisomal disorders: A newly recognised group of genetic diseases.Eur. J. Pediatr. 144 (1986a) 430–440
Schutgens, R. B. H., Romeyn, G-J., Ofman, R., van den Bosch, H., Tager, J. M. and Wanders, R. J. A. Acyl-CoA: dihydroxyacetone phosphate acyltransferase in human skin fibroblasts: study of the properties using a new assay method.Biochim. Biophys. Acta 879 (1986b) 286–291
Schutgens, R. B. H., Wanders, R. J. A., Heymans, H. S. A., Schram, A. W., Tager, J. M., Schrakamp, G. and van den Bosch, H. Zellweger syndrome: biochemical procedures in diagnosis, prevention and treatment.J. Inher. Metab. Dis. 10 Suppl. 1 (1987) 33–45
Schutgens, R. B. H., Heymans, H. S. A., Wanders, R. J. A., Oorthuys, J. W. E., Tager, J. M., Schrakamp, G., van den Bosch, H. and Beemer, F. A. Multiple peroxisomal enzyme deficiencies in rhizomelic chondrodysplasia punctata: comparison with Zellweger syndrome, Conradi-Hunermann syndrome and the X-linked dominant type of chondrodysplasia punctata. In Moss, D. W., Schmidt, E., Schmidt, F. W. (Eds.)Advances in Clinical Enzymology, Karger, Basel, 6 1988, 57–65
Singh, I., Moser, A. B., Goldfischer, S. and Moser, H. W. Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.Proc. Natl. Acad. Sci. USA 81 (1984) 4203–4207
Singh, H., Derwas, N. and Poulos, A. Very long chain fatty acid β-oxidation by rat liver mitochondria and peroxisomes.Arch. Biochem. Biophys. 259 (1987) 382–390
Skjeldal, O. H., Stokke, O., Refsum, S., Norseth, J. and Petit, H. Clinical and biochemical heterogeneity in conditions with phytanic acid accumulation.J. Neurol. Sci. 77 (1987) 87–96
Solish, G. I., Moser, H. W., Ringer, L. D., Moser, A. E., Tiffany, C. and Schutta, E. The prenatal diagnosis of the cerebro-hepato-renal syndrome of Zellweger.Prenat. Diagn. 5 (1985) 27–34
Spranger, J. W., Optiz, J. M. and Bidder, U. Heterogeneity of chondrodysplasia punctata.Hum. Genet. 11 (1971) 190–212
Stellaard, F., Langelaar, S. A., Kok, R. M., Kleijer, W. J., Schutgens, R. B. H. and Jakobs, C. Prenatal diagnosis of Zellweger syndrome by determination of trihydroxycoprostanoic acid in amniotic fluid.26th SSIEM Annual Symposium, Glasgow, P129 (Abstr.)
Suzuki, Y., Orii, T., Takiguchi, M., Mori, M., Hijikata, M. and Hashimoto, T. Biosynthesis of membrane polypeptides of rat liver peroxisomes.J. Biochem. 101 (1987) 491–496
Suzuki, Y., Shimozawa, N., Orii, T., Igarashi, N., Kono, N., Matsui, A., Inoue, Y., Yokata, S. and Hashimoto, T. Zellweger-like syndrome with detectable hepatic peroxisomes: A variant form of peroxisomal disorder.J. Pediatr. 113 (1988) 841–845
Thompson, S. L., Burrow, R., Laub, R. J. and Krisans, S. K. Cholesterol synthesis in rat liver peroxisomes.J. Biol. Chem. 262 (1987) 17420–17425
Tönshoff, B., Lehnert, W. and Ropers, H. H. Adrenoleukodystrophy: diagnosis and carrier detection by determination of very long chain fatty acids in cultured fibroblasts.Clin. Genet. 22 (1982) 25–29
Wanders, R. J. A., Kos, M., Roest, B., Meijer, A. J., Schrakamp, G., Heymans, H. S. A., Tegelaers, W. H. H., van den Bosch, H., Schutgens, R. B. H. and Tager, J. M. Activity of peroxisomal enzymes and intracellular distribution of catalase in Zellweger syndrome.Biochem. Biophys. Res. Commun. 123 (1984) 1054–1061
Wanders, R. J. A., Schrakamp, G., van den Bosch, H., Tager, J. M. and Schutgens, R. B. H. A prenatal test for the cerebro-hepato-renal (Zellweger) syndrome by demonstration of the absence of catalase-containing particles (peroxisomes) in cultured amniotic fluid cells.Eur. J. Pediatr. 145 (1986) 136–138
Wanders, R. J. A., van Roermund, C. W. T., van Wijland, M. J. A., Heikoop, J., Schutgens, R. B. H., Schram, A. W., van den Bosch, H., Poll-Thé, B. T., Saudubray, J. M., Moser, H. W. and Moser, A. B. Peroxisomal very long chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders.Clin. Chim. Acta 166 (1987a) 255–263
Wanders, R. J. A., van Roermund, C. W. T., Westra, R., Schutgens, R. B. H., van de Ende, M. A., Tager, J. M., Monnens, L. A. H., Baadenhuysen, H., Govaerts, L., Przyrembel, H., Wolff, E. D., Blom, W., Huymans, J. G. M. and Laerhoven, F. G. M. Alanine glyoxylate aminotransferase and the urinary excretion of oxalate and glycollate in hyperoxaluria type I and the Zellweger syndrome.Clin. Chim. Acta 165 (1987b) 311–319
Wanders, R. J. A., van Wijland, M. J. A., van Roermund, C. W. T., Schutgens, R. B. H., van den Bosch, H., Tager, J. M., Nijenhuis, A. and Tromp, A. Prenatal diagnosis of Zellweger syndrome by measurement of very long chain fatty acid (C26:0) β-oxidation in cultured chorionic villus fibroblasts: implications for early diagnosis of other peroxisomal disorders.Clin. Chim. Acta 165 (1987c) 303–310
Wanders, R. J. A., van Roermund, C. W. T., van Wijland, M. J. A., Schutgens, R. B. H., van den Bosch, H. and Tager, J. M. 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. 153 (1988a) 618–624
Wanders, R. J. A., Romeyn, G. J., van Roermund, C. W. T., Schutgens, R. B. H., van den Bosch, H. and Tager, J. M. Identification ofl-pipecolic oxidase in human liver and its deficiency in the Zellweger syndrome.Biochem. Biophys. Res. Commun. 154 (1988b) 33–38
Wanders, R. J. A., van Roermund, C. W. T., Jurriaans, S. Schutgens, R. B. H., Tager, J. M., van den Bosch, H., Wolff, E. D., Przyrembel, H., Berger, H., Schaaphok, F. G., Reitsman, W. and van Luyk, W. H. J. Diversity in residual alanine glyoxylate aminotransferase activity in hyperoxaluria type I: Correlation with pyridoxine responsiveness.J. Inher. Metab. Dis. 11 Suppl. 2 (1988c) 208–211
Wanders, R. J. A., Wiemer, E. A. C., Brul, S., Schutgens, R. B. H., van den Bosch, H. and Tager, J. M. Prenatal diagnosis of Zellweger syndrome via a method directly visualizing peroxisomes in chorionic villus fibroblasts and amniocytes.26th SSIEM Annual Symposium, 1988d, Abstract, p. 163
Wanders, R. J. A., Heymans, H. S. A., Schutgens, R. B. H., Barth, P. G., van den Bosch, H. and Tager, J. M. Peroxisomal disorders in neurology.J. Neurol. Sci. 88 (1988e) 1–39
Watkins, P. A., Chen, W. W., Harris, C. J., Hoefler, G., Hoefler, G., Hoefler, S., Blake, Jr., D. S., Balfe, A., Kelley, R. I., Moser, A. B., Beard, M. E. and Moser, H. W. Peroxisomal bifunctional enzyme deficiency.J. Clin. Invest. 83 (1989) 771–777
Williams, H. E. and Smith, L. H. Primary hyperoxaluria. In Stanbury, J. B., Wijngaarden, J. B., Fredrickson, D. S., Goldstein, J. L. and Brown, M. S. (Eds.),The Metabolic Basis of Inherited Disease, 5th edn. McGraw-Hill, New York, 1983, 204–228
Wilson, G. N., Holmes, R. G., Custer, J., Lipkowitz, J. L., Stover, J., Datta, N. and Hajra, A. Zellweger syndrome: Diagnostic assays, syndrome delineation and potential therapy.Am. J. Med. Genet. 24 (1986) 69–82
Yamada, J., Itoh, S., Horie, S., Watanabe, T. and Suga, T. Chain-shortening of a xenobiotic acyl compound by the peroxisomal β-oxidation system in rat liver.Biochem. Pharmacol. 35 (1986) 4363–4368
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Schutgens, R.B.H., Schrakamp, G., Wanders, R.J.A. et al. Prenatal and perinatal diagnosis of peroxisomal disorders. J Inherit Metab Dis 12 (Suppl 1), 118–134 (1989). https://doi.org/10.1007/BF01799291
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DOI: https://doi.org/10.1007/BF01799291