Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 7-Day Trial for You or Your Team.

Learn More →

Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects induced by peroxisome deficiency

Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects... The form and circuitry of the cerebellum develops by a complex process that requires integration of afferent–target interactions between multiple neuronal populations and migratory patterns established by neuron–glial interactions. Analysis of mice lacking the PEX2 peroxisome assembly gene, in which peroxisomal function is disrupted, reveals abnormal cerebellar histogenesis due to the disturbance of multiple cellular processes within neurons. Defects in cerebellar growth and the rostro‐caudal foliation pattern reflect a reduced granule neuron population and abnormal Purkinje cell dendrite development. In granule neurons, there is increased apoptotic cell death and delayed movement from the EGL to IGL that reflects cell cycle, maturational and migrational abnormalities. The underlying Purkinje cells have stunted dendrite arbors with abnormal branching patterns, which may reflect altered inductive influences from the delayed granule neuron translocation. A delayed arborization of mutant olivary climbing fibers and their defective translocation from the perisomatic to the dendritic compartment of Purkinje cells results in numerous spines on the soma and proximal dendrites of Purkinje cells. Distal Purkinje cell dendritic spines also display abnormal morphology. These Purkinje cell dendritic abnormalities are seen in association with persistent and enlarged axonal spheroids, further indicating the presence of a degenerative process within the Purkinje cell. This PEX2−/− mouse model for the human peroxisomal biogenesis disorder Zellweger syndrome illustrates the complex interplay of abnormal developmental processes in the cerebellum and the importance of peroxisomal function for neuronal migration, proliferation, differentiation, and survival. J. Comp. Neurol. 461:394–413, 2003. © 2003 Wiley‐Liss, Inc. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png The Journal of Comparative Neurology Wiley

Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects induced by peroxisome deficiency

Faust, Phyllis L.
The Journal of Comparative Neurology , Volume 461 (3) – Jun 30, 2005
Download PDF
20 pages

Loading next page...
 
/lp/wiley/abnormal-cerebellar-histogenesis-in-pex2-zellweger-mice-reflects-YqRgKrAYYQ

References (83)

Publisher
Wiley
Copyright
"Copyright © 2003 Wiley Subscription Services, Inc., A Wiley Company"
ISSN
0021-9967
eISSN
1096-9861
DOI
10.1002/cne.10699
pmid
12746876
Publisher site
See Article on Publisher Site

Abstract

The form and circuitry of the cerebellum develops by a complex process that requires integration of afferent–target interactions between multiple neuronal populations and migratory patterns established by neuron–glial interactions. Analysis of mice lacking the PEX2 peroxisome assembly gene, in which peroxisomal function is disrupted, reveals abnormal cerebellar histogenesis due to the disturbance of multiple cellular processes within neurons. Defects in cerebellar growth and the rostro‐caudal foliation pattern reflect a reduced granule neuron population and abnormal Purkinje cell dendrite development. In granule neurons, there is increased apoptotic cell death and delayed movement from the EGL to IGL that reflects cell cycle, maturational and migrational abnormalities. The underlying Purkinje cells have stunted dendrite arbors with abnormal branching patterns, which may reflect altered inductive influences from the delayed granule neuron translocation. A delayed arborization of mutant olivary climbing fibers and their defective translocation from the perisomatic to the dendritic compartment of Purkinje cells results in numerous spines on the soma and proximal dendrites of Purkinje cells. Distal Purkinje cell dendritic spines also display abnormal morphology. These Purkinje cell dendritic abnormalities are seen in association with persistent and enlarged axonal spheroids, further indicating the presence of a degenerative process within the Purkinje cell. This PEX2−/− mouse model for the human peroxisomal biogenesis disorder Zellweger syndrome illustrates the complex interplay of abnormal developmental processes in the cerebellum and the importance of peroxisomal function for neuronal migration, proliferation, differentiation, and survival. J. Comp. Neurol. 461:394–413, 2003. © 2003 Wiley‐Liss, Inc.

Journal

The Journal of Comparative Neurology – Wiley

Published: Jun 30, 2005

Keywords: ; ; ; ; ; ;

There are no references for this article.