Aberrant peroxisome morphology in peroxisomal beta-oxidation enzyme deficiencies.
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.
Funato
Michinori
M
Department of Pediatrics, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu 501-1194, Japan. mfunato@mac.com
Shimozawa
Nobuyuki
N
Nagase
Tomoko
T
Takemoto
Yasuhiko
Y
Suzuki
Yasuyuki
Y
Imamura
Yoshihiko
Y
Matsumoto
Tadashi
T
Tsukamoto
Toshiro
T
Kojidani
Tomoko
T
Osumi
Takashi
T
Fukao
Toshiyuki
T
Kondo
Naomi
N
D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency; D042962:Acyl-CoA Oxidase / Q000172:deficiency; D000328:Adult; D002478:Cells, Cultured; D002675:Child, Preschool; D004252:DNA Mutational Analysis / Q000379:methods; D004746:Enoyl-CoA Hydratase / Q000172:deficiency; D005347:Fibroblasts / Q000166:cytology*; D005455:Fluorescent Antibody Technique / Q000379:methods; D006801:Humans; D006836:Hydro-Lyases / Q000172:deficiency; D007223:Infant; D007535:Isomerases / Q000172:deficiency; D046529:Microscopy, Electron, Transmission / Q000379:methods; D009097:Multienzyme Complexes / Q000172:deficiency; D063994:Peroxisomal Bifunctional Enzyme; D018901:Peroxisomal Disorders / Q000201:enzymology* / Q000473:pathology*; D020675:Peroxisomes / Q000201:enzymology* / Q000473:pathology* / Q000648:ultrastructure
D009097:Multienzyme Complexes; D015094:3-Hydroxyacyl CoA Dehydrogenases; D042962:Acyl-CoA Oxidase; C065185:D-3-hydroxyacyl CoA dehydratase; D006836:Hydro-Lyases; C576253:EHHADH protein, human; D004746:Enoyl-CoA Hydratase; D063994:Peroxisomal Bifunctional Enzyme; D007535:Isomerases