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

Author
Organization
Abstract
We report very large hepatic peroxisomes (d-circle > 1-mu-m) 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.
Keywords
PSEUDO-NEONATAL ADRENOLEUKODYSTROPHY, RAT-LIVER, NEONATAL ADRENOLEUKODYSTROPHY, THIOLASE, CATALASE, MORPHOMETRY, CYTOCHEMISTRY, HEPATIC PEROXISOMES, MITOCHONDRIA

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Chicago
De Craemer, Dirk, MJ Zweens, S Lyonnet, RJA Wanders, BT Poll-The, RBH Schutgens, JJJ Waelkens, JM Saudubray, and Frank Roels. 1991. “Very Large Peroxisomes in Distinct Peroxisomal Disorders (rhizomelic Chondrodysplasia Punctata and acyl-CoA Oxidase Deficiency) : Novel Data.” Virchows Archiv A-pathological Anatomy and Histopathology 419 (6): 523–525.
APA
De Craemer, D., Zweens, M., Lyonnet, S., Wanders, R., Poll-The, B., Schutgens, R., Waelkens, J., et al. (1991). Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency) : novel data. VIRCHOWS ARCHIV A-PATHOLOGICAL ANATOMY AND HISTOPATHOLOGY, 419(6), 523–525.
Vancouver
1.
De Craemer D, Zweens M, Lyonnet S, Wanders R, Poll-The B, Schutgens R, et al. Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency) : novel data. VIRCHOWS ARCHIV A-PATHOLOGICAL ANATOMY AND HISTOPATHOLOGY. 1991;419(6):523–5.
MLA
De Craemer, Dirk, MJ Zweens, S Lyonnet, et al. “Very Large Peroxisomes in Distinct Peroxisomal Disorders (rhizomelic Chondrodysplasia Punctata and acyl-CoA Oxidase Deficiency) : Novel Data.” VIRCHOWS ARCHIV A-PATHOLOGICAL ANATOMY AND HISTOPATHOLOGY 419.6 (1991): 523–525. Print.
@article{887272,
  abstract     = {We report very large hepatic peroxisomes (d-circle {\textrangle} 1-mu-m) 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, Dirk and Zweens, MJ and Lyonnet, S and Wanders, RJA and Poll-The, BT and Schutgens, RBH and Waelkens, JJJ and Saudubray, JM and Roels, Frank},
  issn         = {0174-7398},
  journal      = {VIRCHOWS ARCHIV A-PATHOLOGICAL ANATOMY AND HISTOPATHOLOGY},
  language     = {eng},
  number       = {6},
  pages        = {523--525},
  title        = {Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency) : novel data},
  url          = {http://dx.doi.org/10.1007/BF01650683},
  volume       = {419},
  year         = {1991},
}

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