Aberrant peroxisome morphology in peroxisomal beta-oxidation enzyme deficiencies

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• 作者：Michinori Funato ; Nobuyuki Shimozawa ; Tomoko Nagase ; Yasuhiko Takemoto ; Yasuyuki Suzuki ; Yoshihiko Imamura ; Tadashi Matsumoto ; Toshiro Tsukamoto ; Tomoko Kojidani ; Takashi Osumi et al.
• 关键词：Peroxisome ; AOX deficiency ; D-BP deficiency ; Morphology ; Electron microscopy
• 刊名：Brain and Development
• 出版年：2006
• 出版时间：June 2006
• 年：2006
• 卷：28
• 期：5
• 页码：287-292
• 全文大小：366 K

文摘

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 ghostsȁ9; 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.