Aberrant peroxisome morphology in peroxisomal beta-oxidation enzyme deficiencies.

Abstract

Peroxisomes are ubiquitous organelles in eukaryotic cells and surrounded by a single membrane, and undergo considerable changes in size, shape and number. Peroxisomal disorders are classified into two categories: peroxisome biogenesis disorders (PBDs) and single-enzyme deficiencies (SEDs). Morphologically aberrant peroxisomes called 'peroxisomal ghosts' in PBDs are well known, however, a morphological approach to the study of peroxisomes in SEDs has been rarely reported. Here, we investigated the morphology of peroxisomes in cultured fibroblasts from patients lacking peroxisomal beta-oxidation enzymes, including acyl-CoA oxidase (AOX) or D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein (D-BP). Morphological analysis by immunofluorescence examination using an antibody against catalase revealed a smaller number of large peroxisomes in fibroblasts from these patients. Moreover, immunoelectron microscopy using an antibody against the 70-kDa peroxisomal membrane protein (PMP70) showed large peroxisomes with various horseshoe-shaped membrane structures. These results give an important clue to elucidating the division of peroxisomes and how peroxisomes change in size, shape, number and position within cells, which are subjects for future study.

• Authors: Michinori Funato, Nobuyuki Shimozawa, Tomoko Nagase, Yasuhiko Takemoto, Yasuyuki Suzuki, Yoshihiko Imamura, Tadashi Matsumoto, Toshiro Tsukamoto, Tomoko Kojidani, Takashi Osumi, Toshiyuki Fukao, Naomi Kondo
• Journal: Brain & development (Brain Dev) Vol. 28 Issue 5 Pg. 287-92 (Jun 2006) ISSN: 0387-7604 [Print] Netherlands
• PMID: 16376506 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Multienzyme Complexes
• 3-Hydroxyacyl CoA Dehydrogenases
• Acyl-CoA Oxidase
• D-3-hydroxyacyl CoA dehydratase
• Hydro-Lyases
• EHHADH protein, human
• Enoyl-CoA Hydratase
• Peroxisomal Bifunctional Enzyme
• Isomerases

Topics

• 3-Hydroxyacyl CoA Dehydrogenases (deficiency)
• Acyl-CoA Oxidase (deficiency)
• Adult
• Cells, Cultured
• Child, Preschool
• DNA Mutational Analysis (methods)
• Enoyl-CoA Hydratase (deficiency)
• Fibroblasts (cytology)
• Fluorescent Antibody Technique (methods)
• Humans
• Hydro-Lyases (deficiency)
• Infant
• Isomerases (deficiency)
• Microscopy, Electron, Transmission (methods)
• Multienzyme Complexes (deficiency)
• Peroxisomal Bifunctional Enzyme
• Peroxisomal Disorders (enzymology, pathology)
• Peroxisomes (enzymology, pathology, ultrastructure)