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354 related items for PubMed ID: 2538146

  • 1. Adrenoleukodystrophy. The chain shortening of erucic acid (22:1(n-9)) and adrenic acid (22:4(n-6)) is deficient in neonatal adrenoleukodystrophy and normal in X-linked adrenoleukodistrophy skin fibroblasts.
    Christensen E, Grønn M, Hagve TA, Kase BF, Christophersen BO.
    Biochim Biophys Acta; 1989 Mar 14; 1002(1):79-83. PubMed ID: 2538146
    [Abstract] [Full Text] [Related]

  • 2. 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 Mar 14; 215():61-74. PubMed ID: 8327852
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  • 3. Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal beta-oxidation.
    Street JM, Singh H, Poulos A.
    Biochem J; 1990 Aug 01; 269(3):671-7. PubMed ID: 2117919
    [Abstract] [Full Text] [Related]

  • 4. 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 01; 85(20):7647-51. PubMed ID: 3174658
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  • 5. The Zellweger syndrome: deficient chain-shortening of erucic acid (22:1 (n-9)) and adrenic acid (22:4 (n-6)) in cultured skin fibroblasts.
    Christensen E, Hagve TA, Christophersen BO.
    Biochim Biophys Acta; 1988 Mar 25; 959(2):134-42. PubMed ID: 3349094
    [Abstract] [Full Text] [Related]

  • 6. 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 25; 84(5):1425-8. PubMed ID: 3469675
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  • 9. Localization of nervonic acid beta-oxidation in human and rodent peroxisomes: impaired oxidation in Zellweger syndrome and X-linked adrenoleukodystrophy.
    Sandhir R, Khan M, Chahal A, Singh I.
    J Lipid Res; 1998 Nov 25; 39(11):2161-71. PubMed ID: 9799802
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  • 10. 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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  • 11. Cellular oxidation of lignoceric acid is regulated by the subcellular localization of lignoceroyl-CoA ligases.
    Lazo O, Contreras M, Yoshida Y, Singh AK, Stanley W, Weise M, Singh I.
    J Lipid Res; 1990 Apr 05; 31(4):583-95. PubMed ID: 2141053
    [Abstract] [Full Text] [Related]

  • 12. 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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  • 13. 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 01; 29(5):397-408. PubMed ID: 2427264
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  • 14. Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.
    Singh I, Moser AE, Goldfischer S, Moser HW.
    Proc Natl Acad Sci U S A; 1984 Jul 01; 81(13):4203-7. PubMed ID: 6588384
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  • 16. Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy.
    Kemp S, Valianpour F, Denis S, Ofman R, Sanders RJ, Mooyer P, Barth PG, Wanders RJ.
    Mol Genet Metab; 2005 Feb 01; 84(2):144-51. PubMed ID: 15670720
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  • 17. Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction.
    Wiesinger C, Kunze M, Regelsberger G, Forss-Petter S, Berger J.
    J Biol Chem; 2013 Jun 28; 288(26):19269-79. PubMed ID: 23671276
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  • 18. Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes.
    Singh I, Moser AE, Moser HW, Kishimoto Y.
    Pediatr Res; 1984 Mar 28; 18(3):286-90. PubMed ID: 6728562
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  • 19. 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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  • 20. Adrenoleukodystrophy: very long-chain fatty acid metabolism in fibroblasts.
    Rizzo WB, Avigan J, Chemke J, Schulman JD.
    Neurology; 1984 Feb 04; 34(2):163-9. PubMed ID: 6538003
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