Literature DB >> 1750197

Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency): novel data.

D De Craemer1, M J Zweens, S Lyonnet, R J Wanders, B T Poll-The, R B Schutgens, J J Waelkens, J M Saudubray, F Roels.   

Abstract

We report very large hepatic peroxisomes (d-circle greater than 1 micron) in a patient with rhizomelic chondrodysplasia punctata and a patient with acyl-CoA oxidase deficiency. The effects of peroxisomal enlargement on the enzymatic activity are discussed. As increase in peroxisomal size is also reported in at least 12 other patients with peroxisomal disorders, we propose a relationship between the enlargement of the organelles and their functional deficiency.

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Year:  1991        PMID： 1750197     DOI： 10.1007/bf01650683

Source DB:  PubMed          Journal:  Virchows Arch A Pathol Anat Histopathol        ISSN： 0174-7398


  15 in total

1.  Altered acyl-CoA metabolism in riboflavin deficiency.

Authors:  K Veitch; J P Draye; J Vamecq; A G Causey; K Bartlett; H S Sherratt; F Van Hoof
Journal:  Biochim Biophys Acta       Date:  1989-12-18

2.  Light microscopic visualization of peroxisomes and plasmalogens in first trimester chorionic villi.

Authors:  F Roels; V Verdonck; M Pauwels; L De Catte; W Lissens; I Liebaers; M Elleder
Journal:  Prenat Diagn       Date:  1987-09       Impact factor: 3.050

3.  Heterogeneity of catalase staining in human hepatocellular peroxisomes.

Authors:  F Roels; A Cornelis
Journal:  J Histochem Cytochem       Date:  1989-03       Impact factor: 2.479

4.  Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders.

Authors:  J L Hughes; A Poulos; E Robertson; C W Chow; L J Sheffield; J Christodoulou; R F Carter
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1990

5.  Clinical variation in X-linked adrenoleukodystrophy: fatty acid and lipid metabolism in cultured fibroblasts.

Authors:  D J Boles; D A Craft; D A Padgett; R M Loria; W B Rizzo
Journal:  Biochem Med Metab Biol       Date:  1991-02

6.  Effects of marine oil diet on peroxisomes and mitochondria of rat liver. A combined biochemical and morphometric study.

Authors:  E N Christiansen; T Flatmark; H Kryvi
Journal:  Eur J Cell Biol       Date:  1981-12       Impact factor: 4.492

7.  Rhizomelic chondrodysplasia punctata: clinical, pathologic, and biochemical findings in two patients.

Authors:  A Poulos; L Sheffield; P Sharp; G Sherwood; D Johnson; K Beckman; A J Fellenberg; J E Wraith; C W Chow; S Usher
Journal:  J Pediatr       Date:  1988-10       Impact factor: 4.406

8.  Hepatic peroxisomes in adrenoleukodystrophy and related syndromes: cytochemical and morphometric data.

Authors:  F Roels; M Pauwels; B T Poll-Thé; J Scotto; H Ogier; P Aubourg; J M Saudubray
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1988

9.  A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy).

Authors:  B T Poll-The; F Roels; H Ogier; J Scotto; J Vamecq; R B Schutgens; R J Wanders; C W van Roermund; M J van Wijland; A W Schram
Journal:  Am J Hum Genet       Date:  1988-03       Impact factor: 11.025

10.  Neonatal seizures and retardation in a girl with biochemical features of X-linked adrenoleukodystrophy: a possible new peroxisomal disease entity.

Authors:  S Naidu; G Hoefler; P A Watkins; W W Chen; A B Moser; S Hoefler; N E Rance; J M Powers; M Beard; W R Green
Journal:  Neurology       Date:  1988-07       Impact factor: 9.910
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  7 in total

Review 1.  Practical guide for morphometry of human peroxisomes on electron micrographs.

Authors:  I Kerckaert; D De Craemer; G Van Limbergen
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

Review 2.  Secondary alterations of human hepatocellular peroxisomes.

Authors:  D De Craemer
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

3.  Isolated dihydroxyacetonephosphate-acyl-transferase deficiency in rhizomelic chondrodysplasia punctata: clinical presentation, metabolic and histological findings.

Authors:  H Hebestreit; R J Wanders; R B Schutgens; M Espeel; I Kerckaert; F Roels; B Schmausser; L Schrod; A Marx
Journal:  Eur J Pediatr       Date:  1996-12       Impact factor: 3.183

4.  Establishment of a normal range of morphometric values for peroxisomes in paediatric liver.

Authors:  J L Hughes; A J Bourne; A Poulos
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1993

5.  The Roles of β-Oxidation and Cofactor Homeostasis in Peroxisome Distribution and Function in Arabidopsis thaliana.

Authors:  Mauro A Rinaldi; Ashish B Patel; Jaeseok Park; Koeun Lee; Lucia C Strader; Bonnie Bartel
Journal:  Genetics       Date:  2016-09-07       Impact factor: 4.562

6.  Morphometry of peroxisomes and immunolocalization of peroxisomal proteins in the liver of patients with generalised peroxisomal disorders.

Authors:  J L Hughes; D I Crane; E Robertson; A Poulos
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1993

Review 7.  Liver pathology and immunocytochemistry in congenital peroxisomal diseases: a review.

Authors:  F Roels; M Espeel; D De Craemer
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982
  7 in total

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