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253 related items for PubMed ID: 11161822

  • 1. Zellweger syndrome knockout mouse models challenge putative peroxisomal beta-oxidation involvement in docosahexaenoic acid (22:6n-3) biosynthesis.
    Infante JP, Huszagh VA.
    Mol Genet Metab; 2001 Jan; 72(1):1-7. PubMed ID: 11161822
    [Abstract] [Full Text] [Related]

  • 2. Straight-chain acyl-CoA oxidase knockout mouse accumulates extremely long chain fatty acids from alpha-linolenic acid: evidence for runaway carousel-type enzyme kinetics in peroxisomal beta-oxidation diseases.
    Infante JP, Tschanz CL, Shaw N, Michaud AL, Lawrence P, Brenna JT.
    Mol Genet Metab; 2002 Feb; 75(2):108-19. PubMed ID: 11855929
    [Abstract] [Full Text] [Related]

  • 3. Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse).
    Baumgart E, Vanhorebeek I, Grabenbauer M, Borgers M, Declercq PE, Fahimi HD, Baes M.
    Am J Pathol; 2001 Oct; 159(4):1477-94. PubMed ID: 11583975
    [Abstract] [Full Text] [Related]

  • 4. Docosahexaenoic acid membrane content and mRNA expression of acyl-CoA oxidase and of peroxisome proliferator-activated receptor-delta are modulated in Y79 retinoblastoma cells differently by low and high doses of alpha-linolenic acid.
    Langelier B, Furet JP, Perruchot MH, Alessandri JM.
    J Neurosci Res; 2003 Oct 01; 74(1):134-41. PubMed ID: 13130515
    [Abstract] [Full Text] [Related]

  • 5. A review of morphological techniques for detection of peroxisomal (and mitochondrial) proteins and their corresponding mRNAs during ontogenesis in mice: application to the PEX5-knockout mouse with Zellweger syndrome.
    Baumgart E, Fahimi HD, Steininger H, Grabenbauer M.
    Microsc Res Tech; 2003 Jun 01; 61(2):121-38. PubMed ID: 12740819
    [Abstract] [Full Text] [Related]

  • 6. The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction.
    Faust PL, Su HM, Moser A, Moser HW.
    J Mol Neurosci; 2001 Jun 01; 16(2-3):289-97; discussion 317-21. PubMed ID: 11478384
    [Abstract] [Full Text] [Related]

  • 7. Identification of the peroxisomal beta-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid.
    Ferdinandusse S, Denis S, Mooijer PA, Zhang Z, Reddy JK, Spector AA, Wanders RJ.
    J Lipid Res; 2001 Dec 01; 42(12):1987-95. PubMed ID: 11734571
    [Abstract] [Full Text] [Related]

  • 8. Peroxisomal straight-chain Acyl-CoA oxidase and D-bifunctional protein are essential for the retroconversion step in docosahexaenoic acid synthesis.
    Su HM, Moser AB, Moser HW, Watkins PA.
    J Biol Chem; 2001 Oct 12; 276(41):38115-20. PubMed ID: 11500517
    [Abstract] [Full Text] [Related]

  • 9. A mouse model for Zellweger syndrome.
    Baes M, Gressens P, Baumgart E, Carmeliet P, Casteels M, Fransen M, Evrard P, Fahimi D, Declercq PE, Collen D, van Veldhoven PP, Mannaerts GP.
    Nat Genet; 1997 Sep 12; 17(1):49-57. PubMed ID: 9288097
    [Abstract] [Full Text] [Related]

  • 10. Mass spectrometric analysis of ceramide perturbations in brain and fibroblasts of mice and human patients with peroxisomal disorders.
    Pettus BJ, Baes M, Busman M, Hannun YA, Van Veldhoven PP.
    Rapid Commun Mass Spectrom; 2004 Sep 12; 18(14):1569-74. PubMed ID: 15282781
    [Abstract] [Full Text] [Related]

  • 11. Plasmalogens participate in very-long-chain fatty acid-induced pathology.
    Brites P, Mooyer PA, El Mrabet L, Waterham HR, Wanders RJ.
    Brain; 2009 Feb 12; 132(Pt 2):482-92. PubMed ID: 19022859
    [Abstract] [Full Text] [Related]

  • 12. The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation.
    Baes M, Gressens P, Huyghe S, De NK, Qi C, Jia Y, Mannaerts GP, Evrard P, Van VP, Declercq PE, Reddy JK.
    J Neuropathol Exp Neurol; 2002 Apr 12; 61(4):368-74. PubMed ID: 11939592
    [Abstract] [Full Text] [Related]

  • 13. Quantitative analysis of peroxisomal targeting signal type-1 binding to wild-type and pathogenic mutants of Pex5p supports an affinity threshold for peroxisomal protein targeting.
    Maynard EL, Berg JM.
    J Mol Biol; 2007 May 18; 368(5):1259-66. PubMed ID: 17399738
    [Abstract] [Full Text] [Related]

  • 14. Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance.
    Nguyen T, Bjorkman J, Paton BC, Crane DI.
    J Cell Sci; 2006 Feb 15; 119(Pt 4):636-45. PubMed ID: 16449325
    [Abstract] [Full Text] [Related]

  • 15. Genotype-phenotype correlation in PEX5-deficient peroxisome biogenesis defective cell lines.
    Ebberink MS, Mooyer PA, Koster J, Dekker CJ, Eyskens FJ, Dionisi-Vici C, Clayton PT, Barth PG, Wanders RJ, Waterham HR.
    Hum Mutat; 2009 Jan 15; 30(1):93-8. PubMed ID: 18712838
    [Abstract] [Full Text] [Related]

  • 16. Identification of a novel PEX14 mutation in Zellweger syndrome.
    Huybrechts SJ, Van Veldhoven PP, Hoffman I, Zeevaert R, de Vos R, Demaerel P, Brams M, Jaeken J, Fransen M, Cassiman D.
    J Med Genet; 2008 Jun 15; 45(6):376-83. PubMed ID: 18285423
    [Abstract] [Full Text] [Related]

  • 17. Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype.
    Maxwell M, Bjorkman J, Nguyen T, Sharp P, Finnie J, Paterson C, Tonks I, Paton BC, Kay GF, Crane DI.
    Mol Cell Biol; 2003 Aug 15; 23(16):5947-57. PubMed ID: 12897163
    [Abstract] [Full Text] [Related]

  • 18. [Treatment of generalized peroxisomal disorders with docosahexaenoic acid ethyl ether].
    Martínez M, Vázquez E, García-Silva MT, Beltrán JM, Castelló F, Pineda M, Mougan I.
    Rev Neurol; 1999 Jan 15; 28 Suppl 1():S59-64. PubMed ID: 10778491
    [Abstract] [Full Text] [Related]

  • 19. On the molecular etiology of decreased arachidonic (20:4n-6), docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids in Zellweger syndrome and other peroxisomal disorders.
    Infante JP, Huszagh VA.
    Mol Cell Biochem; 1997 Mar 15; 168(1-2):101-15. PubMed ID: 9062899
    [Abstract] [Full Text] [Related]

  • 20. Plasmalogens, phospholipase A2, and docosahexaenoic acid turnover in brain tissue.
    Farooqui AA, Horrocks LA.
    J Mol Neurosci; 2001 Mar 15; 16(2-3):263-72; discussion 279-84. PubMed ID: 11478381
    [Abstract] [Full Text] [Related]

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