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275 related items for PubMed ID: 2049396

  • 1. Immunochemical and biochemical studies of fatty acid oxidation in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients.
    Reubsaet FA, Veerkamp JH, Brückwilder ML, Trijbels JM, Hashimoto T, Monnens LA.
    Biochim Biophys Acta; 1991 Jun 03; 1083(3):305-9. PubMed ID: 2049396
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  • 4. Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.
    Chen WW, Watkins PA, Osumi T, Hashimoto T, Moser HW.
    Proc Natl Acad Sci U S A; 1987 Mar 03; 84(5):1425-8. PubMed ID: 3469675
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  • 5. The deficient degradation of synthetic 2- and 3-methyl-branched fatty acids in fibroblasts from patients with peroxisomal disorders.
    Van Veldhoven PP, Huang S, Eyssen HJ, Mannaerts GP.
    J Inherit Metab Dis; 1993 Mar 03; 16(2):381-91. PubMed ID: 7692128
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  • 6. Transport of fatty acids into human and rat peroxisomes. Differential transport of palmitic and lignoceric acids and its implication to X-adrenoleukodystrophy.
    Singh I, Lazo O, Dhaunsi GS, Contreras M.
    J Biol Chem; 1992 Jul 05; 267(19):13306-13. PubMed ID: 1618832
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  • 7. Peroxisomal beta-oxidation of polyunsaturated long chain fatty acids in human fibroblasts. The polyunsaturated and the saturated long chain fatty acids are retroconverted by the same acyl-CoA oxidase.
    Christensen E, Woldseth B, Hagve TA, Poll-The BT, Wanders RJ, Sprecher H, Stokke O, Christophersen BO.
    Scand J Clin Lab Invest Suppl; 1993 Jul 05; 215():61-74. PubMed ID: 8327852
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  • 8. X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters.
    Wanders RJ, van Roermund CW, van Wijland MJ, Nijenhuis AA, Tromp A, Schutgens RB, Brouwer-Kelder EM, Schram AW, Tager JM, van den Bosch H.
    Clin Chim Acta; 1987 Jun 15; 165(2-3):321-9. PubMed ID: 3652454
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  • 9. Accumulation and defective beta-oxidation of very long chain fatty acids in Zellweger's syndrome, adrenoleukodystrophy and Refsum's disease variants.
    Poulos A, Singh H, Paton B, Sharp P, Derwas N.
    Clin Genet; 1986 May 15; 29(5):397-408. PubMed ID: 2427264
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  • 10. A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy).
    Poll-The BT, Roels F, Ogier H, Scotto J, Vamecq J, Schutgens RB, Wanders RJ, van Roermund CW, van Wijland MJ, Schram AW.
    Am J Hum Genet; 1988 Mar 15; 42(3):422-34. PubMed ID: 2894756
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  • 11. Peroxisomal very long-chain fatty acid beta-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders.
    Wanders RJ, van Roermund CW, van Wijland MJ, Heikoop J, Schutgens RB, Schram AW, Tager JM, van den Bosch H, Poll-Thé BT, Saudubray JM.
    Clin Chim Acta; 1987 Jul 15; 166(2-3):255-63. PubMed ID: 2441904
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  • 12. Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.
    Lazo O, Contreras M, Hashmi M, Stanley W, Irazu C, Singh I.
    Proc Natl Acad Sci U S A; 1988 Oct 15; 85(20):7647-51. PubMed ID: 3174658
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  • 13. Adrenoleukodystrophy: impaired oxidation of fatty acids due to peroxisomal lignoceroyl-CoA ligase deficiency.
    Lazo O, Contreras M, Bhushan A, Stanley W, Singh I.
    Arch Biochem Biophys; 1989 May 01; 270(2):722-8. PubMed ID: 2705786
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  • 14. Profiles of very-long-chain fatty acids in plasma, fibroblasts, and blood cells in Zellweger syndrome, X-linked adrenoleukodystrophy, and rhizomelic chondrodysplasia punctata.
    Schutgens RB, Bouman IW, Nijenhuis AA, Wanders RJ, Frumau ME.
    Clin Chem; 1993 Aug 01; 39(8):1632-7. PubMed ID: 8353949
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  • 15. Very-long-chain polyunsaturated fatty acids accumulate in phosphatidylcholine of fibroblasts from patients with Zellweger syndrome and acyl-CoA oxidase1 deficiency.
    Abe Y, Honsho M, Nakanishi H, Taguchi R, Fujiki Y.
    Biochim Biophys Acta; 2014 Apr 04; 1841(4):610-9. PubMed ID: 24418004
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  • 16. 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.
    J Clin Invest; 1987 Dec 04; 80(6):1778-83. PubMed ID: 3680527
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  • 17. Photosensitized killing of cultured fibroblasts from patients with peroxisomal disorders due to pyrene fatty acid-mediated ultraviolet damage.
    Hoefler G, Paschke E, Hoefler S, Moser AB, Moser HW.
    J Clin Invest; 1991 Dec 04; 88(6):1873-9. PubMed ID: 1752949
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  • 18. Different intracellular localization of peroxisomal proteins in fibroblasts from patients with aberrant peroxisome assembly.
    Suzuki Y, Shimozawa N, Yajima S, Orii T, Yokota S, Tashiro Y, Osumi T, Hashimoto T.
    Cell Struct Funct; 1992 Feb 04; 17(1):1-8. PubMed ID: 1586963
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  • 20. Late-onset generalized disorder of peroxisomes.
    Burdette DE, Kremser K, Fink JK, Pahan K, Stanley W, Singh I.
    Neurology; 1996 Mar 04; 46(3):829-31. PubMed ID: 8618694
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