Title: Generalised and conditional inactivation of Pex genes in mice
Authors: Baes, Myriam ×
Van Veldhoven, Paul P #
Issue Date: Dec-2006
Publisher: Elsevier science bv
Series Title: Biochimica et Biophysica Acta. Molecular Cell Research vol:1763 issue:12 pages:1785-1793
Abstract: During the past 10 years, several Pex genes have been knocked out in the mouse with the purpose to generate models to study the pathogenesis of peroxisome biogenesis disorders and/or to investigate the physiological importance of the Pex proteins. More recently, mice with selective inactivation of a Pex gene in particular cell types were created. The metabolic abnormalities in peroxisome deficient mice paralleled to a large extent those of Zellweger patients. Several but not all of the clinical and histological features reported in patients also occurred in peroxisome deficient mice as for example hypotonia, cortical and cerebellar malformations, endochondral ossification defects, hepatomegaly, liver fibrosis and ultrastructural abnormalities of mitochondria in hepatocytes. Although the molecular origins of the observed pathologies have not yet been resolved, several new insights on the importance of peroxisomes in different tissues have emerged. (c) 2006 Elsevier B.V. All rights reserved.
ISSN: 0167-4889
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Cell Metabolism
Pharmacology Section (-)
Laboratory of Lipid Biochemistry and Protein Interactions
× corresponding author
# (joint) last author

Files in This Item:

There are no files associated with this item.

Request a copy


All items in Lirias are protected by copyright, with all rights reserved.

© Web of science