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

D. de Craemer; M. J. Zweens; S. Lyonnet; R. J. Wanders; B. T. Poll-The; R. B. Schutgens; J. J. Waelkens; J. M. Saudubray; F. Roels

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

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

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Research output: Contribution to journal › Article › Academic › peer-review

16 Citations (Scopus)

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

Original language	English
Pages (from-to)	523-525
Journal	Virchows Archiv. A, Pathological anatomy and histopathology
Volume	419
Issue number	6
Publication status	Published - 1991

Cite this

@article{e5e9742541574479a59b51cc40bda118,

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

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",

author = "{de Craemer}, D. and Zweens, {M. J.} and S. Lyonnet and Wanders, {R. J.} and Poll-The, {B. T.} and Schutgens, {R. B.} and Waelkens, {J. J.} and Saudubray, {J. M.} and F. Roels",

year = "1991",

language = "English",

volume = "419",

pages = "523--525",

journal = "Virchows Archiv. A, Pathological anatomy and histopathology",

issn = "0174-7398",

publisher = "Springer Verlag",

number = "6",

}

Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency): novel data. / de Craemer, D.; Zweens, M. J.; Lyonnet, S. et al.
In: Virchows Archiv. A, Pathological anatomy and histopathology, Vol. 419, No. 6, 1991, p. 523-525.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency): novel data

AU - de Craemer, D.

AU - Zweens, M. J.

AU - Lyonnet, S.

AU - Wanders, R. J.

AU - Poll-The, B. T.

AU - Schutgens, R. B.

AU - Waelkens, J. J.

AU - Saudubray, J. M.

AU - Roels, F.

PY - 1991

Y1 - 1991

N2 - 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

AB - 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

M3 - Article

C2 - 1750197

SN - 0174-7398

VL - 419

SP - 523

EP - 525

JO - Virchows Archiv. A, Pathological anatomy and histopathology

JF - Virchows Archiv. A, Pathological anatomy and histopathology

IS - 6

ER -