Santos M J, Imanaka T, Shio H, Small G M, Lazarow P B
Rockefeller University, New York, NY 10021.
Science. 1988 Mar 25;239(4847):1536-8. doi: 10.1126/science.3281254.
Peroxisomes are apparently missing in Zellweger syndrome; nevertheless, some of the integral membrane proteins of the organelle are present. Their distribution was studied by immunofluorescence microscopy. In control fibroblasts, peroxisomes appeared as small dots. In Zellweger fibroblasts, the peroxisomal membrane proteins were located in unusual empty membrane structures of larger size. These results suggest that the primary defect in this disease may be in the mechanism for import of matrix proteins.
在齐-韦二氏综合征中，过氧化物酶体明显缺失；然而，该细胞器的一些整合膜蛋白却存在。通过免疫荧光显微镜研究了它们的分布。在对照成纤维细胞中，过氧化物酶体呈现为小点状。在齐-韦二氏综合征成纤维细胞中，过氧化物酶体膜蛋白位于较大尺寸的异常空膜结构中。这些结果表明，该疾病的主要缺陷可能在于基质蛋白的导入机制。
