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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.
Brul S, Westerveld A, Strijland A, Wanders RJ, Schram AW, Heymans HS, Schutgens RB, van den Bosch H, Tager JM. Brul S, et al. Among authors: schram aw. J Clin Invest. 1988 Jun;81(6):1710-5. doi: 10.1172/JCI113510. J Clin Invest. 1988. PMID: 2454948 Free PMC article.
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.
Wanders RJ, van Roermund CW, van Wijland MJ, Schutgens RB, Schram AW, Tager JM, van den Bosch H, Schalkwijk C. Wanders RJ, et al. Among authors: schram aw. J Inherit Metab Dis. 1988;11 Suppl 2:173-7. doi: 10.1007/BF01804228. J Inherit Metab Dis. 1988. PMID: 3141702 No abstract available.
Kinetics of the assembly of peroxisomes after fusion of complementary cell lines from patients with the cerebro-hepato-renal (Zellweger) syndrome and related disorders.
Brul S, Wiemer EA, Westerveld A, Strijland A, Wanders RJ, Schram AW, Heymans HS, Schutgens RB, Van den Bosch H, Tager JM. Brul S, et al. Among authors: schram aw. Biochem Biophys Res Commun. 1988 May 16;152(3):1083-9. doi: 10.1016/s0006-291x(88)80395-4. Biochem Biophys Res Commun. 1988. PMID: 3377768
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.
Wanders RJ, van Roermund CW, van Wijland MJ, Schutgens RB, van den Bosch H, Schram AW, Tager JM. Wanders RJ, et al. Among authors: schram aw. Biochem Biophys Res Commun. 1988 Jun 16;153(2):618-24. doi: 10.1016/s0006-291x(88)81140-9. Biochem Biophys Res Commun. 1988. PMID: 3382393
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.
Wanders RJ, van Roermund CW, van Wijland MJ, Schutgens RB, Heikoop J, van den Bosch H, Schram AW, Tager JM. Wanders RJ, et al. Among authors: schram aw. J Clin Invest. 1987 Dec;80(6):1778-83. doi: 10.1172/JCI113271. J Clin Invest. 1987. PMID: 3680527 Free PMC article.
100 results