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Journal Abstract Search

207 related items for PubMed ID: 11992265

  • 1. Reinvestigation of peroxisomal 3-ketoacyl-CoA thiolase deficiency: identification of the true defect at the level of d-bifunctional protein.
    Ferdinandusse S, van Grunsven EG, Oostheim W, Denis S, Hogenhout EM, IJlst L, van Roermund CW, Waterham HR, Goldfischer S, Wanders RJ.
    Am J Hum Genet; 2002 Jun; 70(6):1589-93. PubMed ID: 11992265
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  • 2. 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
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  • 3. D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency: a newly identified peroxisomal disorder.
    Suzuki Y, Jiang LL, Souri M, Miyazawa S, Fukuda S, Zhang Z, Une M, Shimozawa N, Kondo N, Orii T, Hashimoto T.
    Am J Hum Genet; 1997 Nov 12; 61(5):1153-62. PubMed ID: 9345094
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  • 4. Clinical consequences of defects in peroxisomal beta-oxidation.
    Clayton PT.
    Biochem Soc Trans; 2001 May 12; 29(Pt 2):298-305. PubMed ID: 11356171
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  • 5. Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein.
    Jiang LL, Kurosawa T, Sato M, Suzuki Y, Hashimoto T.
    J Biochem; 1997 Mar 12; 121(3):506-13. PubMed ID: 9133619
    [Abstract] [Full Text] [Related]

  • 6. Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency.
    van Grunsven EG, van Berkel E, Ijlst L, Vreken P, de Klerk JB, Adamski J, Lemonde H, Clayton PT, Cuebas DA, Wanders RJ.
    Proc Natl Acad Sci U S A; 1998 Mar 03; 95(5):2128-33. PubMed ID: 9482850
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  • 7. Gene expression of peroxisomal beta-oxidation enzymes in rat brain.
    Knoll A, Sargueil F, Salles J, Cassagne C, Garbay B.
    Brain Res Mol Brain Res; 1999 Dec 10; 74(1-2):217-20. PubMed ID: 10640693
    [Abstract] [Full Text] [Related]

  • 8. Molecular analysis of peroxisomal beta-oxidation enzymes in infants with peroxisomal disorders indicates heterogeneity of the primary defect.
    Guerroui S, Aubourg P, Chen WW, Hashimoto T, Scotto J.
    Biochem Biophys Res Commun; 1989 May 30; 161(1):242-51. PubMed ID: 2471528
    [Abstract] [Full Text] [Related]

  • 9. Developmental and pathological expression of peroxisomal enzymes: their relationship of D-bifunctional protein deficiency and Zellweger syndrome.
    Itoh M, Suzuki Y, Akaboshi S, Zhang Z, Miyabara S, Takashima S.
    Brain Res; 2000 Mar 06; 858(1):40-7. PubMed ID: 10700594
    [Abstract] [Full Text] [Related]

  • 10. Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency.
    van Grunsven EG, Mooijer PA, Aubourg P, Wanders RJ.
    Hum Mol Genet; 1999 Aug 06; 8(8):1509-16. PubMed ID: 10400999
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  • 16. A novel HPLC-based method to diagnose peroxisomal D-bifunctional protein enoyl-CoA hydratase deficiency.
    Gloerich J, Denis S, van Grunsven EG, Dacremont G, Wanders RJ, Ferdinandusse S.
    J Lipid Res; 2003 Mar 06; 44(3):640-4. PubMed ID: 12562856
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  • 17. Peroxisomal localization in the developing mouse cerebellum: implications for neuronal abnormalities related to deficiencies in peroxisomes.
    Nagase T, Shimozawa N, Takemoto Y, Suzuki Y, Komori M, Kondo N.
    Biochim Biophys Acta; 2004 Mar 17; 1671(1-3):26-33. PubMed ID: 15026142
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  • 18. Targeted disruption of the peroxisomal thiolase B gene in mouse: a new model to study disorders related to peroxisomal lipid metabolism.
    Chevillard G, Clémencet MC, Latruffe N, Nicolas-Francès V.
    Biochimie; 2004 Nov 17; 86(11):849-56. PubMed ID: 15589695
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  • 20. Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
    van Grunsven EG, van Berkel E, Mooijer PA, Watkins PA, Moser HW, Suzuki Y, Jiang LL, Hashimoto T, Hoefler G, Adamski J, Wanders RJ.
    Am J Hum Genet; 1999 Jan 17; 64(1):99-107. PubMed ID: 9915948
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