Co-organizersJohn Aitchison, Institute for Systems Biology Richard Rachubinski, University of Alberta Suresh Subramani, University of California San Diego Ralf Erdmann, Ruhr-Universität Bochum Yukio Fujiki, Kyushu University About Peroxisome DisordersDysfunctional peroxisomes have catastrophic consequences leading to conditions that primarily afflict children. Often patients suffer from severe neurological, liver and kidney defects which can be fatal in early childhood. Single Peroxisomal Enzyme Deficiencies:
Peroxisomal Biogenesis Disorders
About PeroxisomesPeroxisomes are ubiquitous intracellular organelles that play many regulated metabolic roles in human cellular physiology including beta-oxidation of long chain fatty acids; the synthesis of cholesterol, bile acids and plasmalogens; and the decomposition of hydrogen peroxide and superoxides. What makes peroxisomes remarkable is that they respond dramatically to various stimuli and their abundance, size, and enzymatic profiles vary by tissue location. Because of their varied metabolic and cellular roles, peroxisomes are linked to a number of human health concerns including aging, neuropathology, cancer, heart disease, obesity, and diabetes. A number of inherited genetic disorders, collectively called the peroxisome biogenesis disorders, are attributed to dysfunction of peroxisome assembly. Understanding the plasticity of peroxisomes, their different functions and the details of their biogenesis is important for determining the contributions the organelle makes to many human disorders and for developing treatments for these conditions.
|
||||||||||||||
![]() |
||||||||||||||
| © 2009, Institute for Systems Biology, All Rights Reserved | ||||||||||||||