Peroxisomal Membrane Ghosts in Zellweger Syndrome—Aberrant Organelle Assembly

作者：M. J. Santos, Tsuneo Imanaka, H Shio, G. M. Small, P. B. Lazarow · 发表于：Science · 年份：1988 · DOI：10.1126/science.3281254 · 被引用次数：328 · 研究领域：Peroxisome Proliferator-Activated Receptors、Genomics, phytochemicals, and oxidative stress、RNA regulation and disease

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.