Development of the human cerebellum and its disorders.
The cerebellum arises from two anatomically and molecularly different proliferative compartments: the cerebellar ventricular zone and the rhombic lip. The protracted development makes the cerebellum vulnerable to a broad spectrum of developmental disorders, of which the more frequent (the Dandy-Walker and related malformations and the pontocerebellar hypoplasias) are discussed in this article. Several genes for congenital malformations of the human cerebellum have recently been identified, including genes causing Joubert syndrome, the Dandy-Walker malformation, and pontocerebellar hypoplasias.
Ten Donkelaar
Hans J
HJ
Department of Neurology, 935, Radboud University Nijmegen Medical Center, Reinier Postlaan 4, 6525 GC, PO Box 9101, 6500 HB Nijmegen, The Netherlands. h.tendonkelaar@neuro.umcn.nl
Lammens
Martin
M
D000818:Animals; D002454:Cell Differentiation / Q000502:physiology; D002465:Cell Movement / Q000502:physiology; D002526:Cerebellar Diseases / Q000235:genetics / Q000473:pathology*; D002531:Cerebellum / Q000002:abnormalities* / Q000196:embryology* / Q000473:pathology*; D006801:Humans; D009421:Nervous System Malformations / Q000235:genetics / Q000473:pathology*