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Volumn 19, Issue 2, 1996, Pages 193-196

Cholesterol biosynthesis in Zellweger syndrome: Normal activity of mevalonate kinase, mevalonate-5′-pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver

Author keywords

[No Author keywords available]

Indexed keywords

CARBOXYLYASE; CHOLESTEROL; IPP ISOMERASE; ISOMERASE; LOW DENSITY LIPOPROTEIN; MEVALONATE 5' PYROPHOSPHATE DECARBOXYLASE; MEVALONIC ACID; PHOSPHOTRANSFERASE; UNCLASSIFIED DRUG;

EID: 0029878455     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF01799427     Document Type: Article
Times cited : (16)

References (13)
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  • 4
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    • 3-Hydroxy-3-methylglutarylcoenzyme A reductase is present in peroxisomes in normal rat liver cells
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    • Keller, G.-A.1    Barton, M.C.2    Shapiro, P.J.3    Singer, S.J.4
  • 5
    • 0026932050 scopus 로고
    • The role of peroxisomes in cholesterol metabolism
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    • (1992) Am J Respir Cell Mol Biol , vol.7 , pp. 358-364
    • Krisans, S.K.1
  • 7
    • 0028944018 scopus 로고
    • Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin
    • Malle E, Oettl K, Sattler W, Hoefler G, Kostner GM (1995) Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin. Eur J Clin Invest 25: 59-67.
    • (1995) Eur J Clin Invest , vol.25 , pp. 59-67
    • Malle, E.1    Oettl, K.2    Sattler, W.3    Hoefler, G.4    Kostner, G.M.5
  • 8
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    • Mandel H, Getsis M, Rosenblat M, Berant M, Aviram M (1995) Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patient's LDL and with reduced cholesterol synthesis. J Lipid Res 36: 1385-1391.
    • (1995) J Lipid Res , vol.36 , pp. 1385-1391
    • Mandel, H.1    Getsis, M.2    Rosenblat, M.3    Berant, M.4    Aviram, M.5
  • 9
    • 0019458732 scopus 로고
    • Control of phosphorylation and decarboxylation of mevalonic acid and its metabolites in cultured human fibroblasts and in rat liver in vivo
    • Mitchell ED, Avigan J (1981) Control of phosphorylation and decarboxylation of mevalonic acid and its metabolites in cultured human fibroblasts and in rat liver in vivo, J Biol Chem 256: 6170-6173.
    • (1981) J Biol Chem , vol.256 , pp. 6170-6173
    • Mitchell, E.D.1    Avigan, J.2
  • 10
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    • Differential protein import deficiencies in human peroxisome assembly disorders
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  • 11
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    • Infantile Refsum disease: An inherited peroxisomal disorder: comparison with Zellweger syndrome and neonatal adrenoleukodystrophy
    • Poll-The BT, Saudubray JM, Ogier HAM, et al (1987) Infantile Refsum disease: an inherited peroxisomal disorder: comparison with Zellweger syndrome and neonatal adrenoleukodystrophy. Eur J Pediatr 146: 477-483.
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    • Poll-The, B.T.1    Saudubray, J.M.2    Ogier, H.A.M.3
  • 12
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    • Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders
    • in press
    • Slawecki ML, Dodt G, Moser AB, Moser HW, Gould SJ (1995) Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders. J Cell Biol, in press.
    • (1995) J Cell Biol
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* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.