Literature DB >> 8739963

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.

R J Wanders1, G J Romeijn.   

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Year:  1996        PMID： 8739963     DOI： 10.1007/bf01799427

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  13 in total

1.  Infantile Refsum disease: an inherited peroxisomal disorder. Comparison with Zellweger syndrome and neonatal adrenoleukodystrophy.

Authors:  B T Poll-The; J M Saudubray; H A Ogier; M Odièvre; J M Scotto; L Monnens; L C Govaerts; F Roels; A Cornelis; R B Schutgens
Journal:  Eur J Pediatr       Date:  1987-09       Impact factor: 3.183

2.  Farnesyl-diphosphate synthase is localized in peroxisomes.

Authors:  S K Krisans; J Ericsson; P A Edwards; G A Keller
Journal:  J Biol Chem       Date:  1994-05-13       Impact factor: 5.157

3.  Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin.

Authors:  E Malle; K Oettl; W Sattler; G Hoefler; G M Kostner
Journal:  Eur J Clin Invest       Date:  1995-01       Impact factor: 4.686

4.  Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders.

Authors:  G Dodt; N Braverman; C Wong; A Moser; H W Moser; P Watkins; D Valle; S J Gould
Journal:  Nat Genet       Date:  1995-02       Impact factor: 38.330

5.  Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patient's low density lipoprotein and with reduced cholesterol synthesis.

Authors:  H Mandel; M Getsis; M Rosenblat; M Berant; M Aviram
Journal:  J Lipid Res       Date:  1995-06       Impact factor: 5.922

6.  Mevalonate kinase is localized in rat liver peroxisomes.

Authors:  K D Stamellos; J E Shackelford; R D Tanaka; S K Krisans
Journal:  J Biol Chem       Date:  1992-03-15       Impact factor: 5.157

7.  3-Hydroxy-3-methylglutaryl-coenzyme A reductase is present in peroxisomes in normal rat liver cells.

Authors:  G A Keller; M C Barton; D J Shapiro; S J Singer
Journal:  Proc Natl Acad Sci U S A       Date:  1985-02       Impact factor: 11.205

8.  Control of phosphorylation and decarboxylation of mevalonic acid and its metabolites in cultured human fibroblasts and in rat liver in vivo.

Authors:  E D Mitchell; J Avigan
Journal:  J Biol Chem       Date:  1981-06-25       Impact factor: 5.157

9.  Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders.

Authors:  M L Slawecki; G Dodt; S Steinberg; A B Moser; H W Moser; S J Gould
Journal:  J Cell Sci       Date:  1995-05       Impact factor: 5.285

10.  Differential protein import deficiencies in human peroxisome assembly disorders.

Authors:  A Motley; E Hettema; B Distel; H Tabak
Journal:  J Cell Biol       Date:  1994-05       Impact factor: 10.539
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  2 in total

1.  Cholesterol biosynthesis, peroxisomes and peroxisomal disorders: mevalonate kinase is not only deficient in Zellweger syndrome but also in rhizomelic chondrodysplasia punctata.

Authors:  R J Wanders; G J Romeijn
Journal:  J Inherit Metab Dis       Date:  1998-06       Impact factor: 4.982

2.  Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model.

Authors:  Werner J Kovacs; Janis E Shackelford; Khanichi N Tape; Michael J Richards; Phyllis L Faust; Steven J Fliesler; Skaidrite K Krisans
Journal:  Mol Cell Biol       Date:  2004-01       Impact factor: 4.272
  2 in total

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