Early postnatal Purkinje cells from staggerer mice undergo aberrant development in vitro with characteristic morphologic and gene expression abnormalities.
The staggerer (sg) mutant mouse shows an early block in Purkinje cell (PC) development due to a truncation of the transcription factor retinoic acid-related orphan receptor alpha (ROR alpha). Cerebellar cultures from neonatal sg mice recapitulate many in vivo postnatal changes in PCs and allow for detailed observation of sequential dendritic outgrowth and gene expression changes. In addition, this in vitro system provides a tractable basis for ROR alpha replacement studies.
Shirley
L Thomas
LT
Department of Biomedical Sciences, University at Albany, Albany, NY 12203, United States.
Messer
Anne
A
D000818:Animals; D000831:Animals, Newborn; D001369:Axons / Q000378:metabolism / Q000473:pathology; D064026:Calbindins; D002454:Cell Differentiation / Q000235:genetics; D048430:Cell Shape / Q000235:genetics; D002478:Cells, Cultured; D002525:Cerebellar Cortex / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology*; D003712:Dendrites / Q000378:metabolism / Q000473:pathology; D018507:Gene Expression Regulation, Developmental / Q000502:physiology*; D020662:Guanine Nucleotide Exchange Factors; D051379:Mice; D008818:Mice, Neurologic Mutants; D009421:Nervous System Malformations / Q000235:genetics*; D009479:Neuropeptides / Q000378:metabolism; D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; D011689:Purkinje Cells / Q000378:metabolism / Q000473:pathology*; D018160:Receptors, Cytoplasmic and Nuclear / Q000235:genetics*; D064030:S100 Calcium Binding Protein G / Q000378:metabolism; D015534:Trans-Activators / Q000235:genetics*
D064026:Calbindins; D020662:Guanine Nucleotide Exchange Factors; D009479:Neuropeptides; D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; C104971:Pcp2 protein, mouse; D018160:Receptors, Cytoplasmic and Nuclear; D064030:S100 Calcium Binding Protein G; D015534:Trans-Activators