The role of endoplastic reticulum in the de novo biogenesis of peroxisomes

真核细胞的一个标志是亚细胞细胞器的多样化排列，这些细胞器划分了生命所必需的各种生化反应。过氧化物酶体就是这样一种细胞器。这个小细胞器容纳酶，酶参与各种生物反应，这些反应对生物体的生存至关重要。例如，过氧化物酶体在人体代谢中的重要性被几种遗传疾病的存在所强调，其中一种或多种过氧化物酶体蛋白存在缺陷。最严重的病例（统称为过氧化物酶体生物发生障碍[PBDs]）是由于细胞不能形成过氧化物酶体造成的，通常表现为身体畸形、神经退行性变以及肝、肾和心脏疾病，最终导致死亡。虽然这些疾病表明过氧化物酶体在基本细胞功能中的重要性，但我们仍然缺乏对过氧化物酶体如何形成的基本了解。因此，我们研究计划的总体目标是利用最先进的成像技术结合生化技术阐明哺乳动物细胞中过氧化物酶体形成的基本机制。这些研究在更广泛的背景下也很重要，因为过氧化物酶体在多细胞生物（如植物）之间的功能是保守的，其中过氧化物酶体在植物生长中起着关键作用。最终，通过这项研究的发现不仅将进一步加深我们对过氧化物酶体形成的理解，而且通过这项研究开发的创新技术也可以用于研究细胞生物学的其他主题。此外，它将为年轻科学家的发展提供培训，为加拿大学术界、政府或工业的职业生涯做好准备。

A hallmark of eukaryotic cells is the diverse array of subcellular organelles that compartmentalize the various biochemical reactions essential to life. One such organelle is the peroxisome. This small organelle houses enzymes, which are involved in a wide variety of biological reactions that are critical for the survival of the organism. For instance, the significance of peroxisomes in human metabolism is underscored by the existence of several genetic disorders in which one or more peroxisomal proteins are defective. The most severe cases (collectively called Peroxisome Biogenesis Disorders [PBDs]) result from the inability of cells to form peroxisomes and are typically characterized by physical deformities, neurodegeneration and diseases of the liver, kidney and heart, that eventually leads to death. Although these diseases signify the importance of peroxisomes in basic cellular function, we still lack a basic understanding of how peroxisomes are formed. Therefore, the overall goal of our research proposal is to elucidate the basic mechanism of peroxisome formation in mammalian cells using state-of-the-art imaging technology coupled with biochemical techniques. These studies will also be important in a broader context since peroxisome function is conserved between multi-cellular organisms such as plants where peroxisomes play a critical role in plant growth. Ultimately, the findings through this research will not only further our understanding of peroxisome formation but the innovative techniques developed through this research can also be utilized to study other topics of cell biology. Furthermore, it will provide training for the development of young scientists who will be well prepared for careers in Canadian academia, government, or industry.