Hughes J L, Poulos A, Crane D I, Chow C W, Sheffield L J, Sillence D
Department of Histopathology, Adelaide Children's Hospital, South Australia.
Eur J Pediatr. 1992 Nov;151(11):829-36. doi: 10.1007/BF01957935.
Peroxisomes were studied in the liver of two rhizomelic chondrodysplasia punctata patients using electron microscopy and catalase cytochemistry. Immunoelectron microscopy was carried out on the liver of one of these patients using antibodies to catalase, acyl-CoA oxidase, bifunctional protein, 3-ketoacyl-CoA thiolase and a 68 kDa peroxisomal membrane protein, in conjunction with protein-A colloidal gold. Moderately to markedly enlarged, flocculent peroxisomes were found in both patients. In one patient they were very heterogeneous with regard to the number per hepatocyte. The peroxisomes had very low levels of catalase as indicated by cytochemistry and immunocytochemistry. The three beta-oxidation enzymes were localised normally within the peroxisomes. The 68 kDa membrane protein was localised to the peroxisomal membranes. Some extra membrane loops were also identified using this antibody.
利用电子显微镜和过氧化氢酶细胞化学方法，对两名点状软骨发育不良（肢根型）患者的肝脏中的过氧化物酶体进行了研究。其中一名患者的肝脏进行了免疫电子显微镜检查，使用了针对过氧化氢酶、酰基辅酶A氧化酶、双功能蛋白、3-酮酰基辅酶A硫解酶和一种68 kDa过氧化物酶体膜蛋白的抗体，并结合蛋白A胶体金。两名患者均发现有中度至明显增大的絮状过氧化物酶体。在一名患者中，每个肝细胞中的过氧化物酶体数量差异很大。细胞化学和免疫细胞化学结果表明，过氧化物酶体中的过氧化氢酶水平非常低。三种β-氧化酶正常定位于过氧化物酶体内。68 kDa膜蛋白定位于过氧化物酶体膜上。使用该抗体还鉴定出了一些额外的膜环。
