Longitudinal brain MRI study in a mouse model of Rett Syndrome and the effects of choline.
Rett Syndrome (RTT), the second most common cause of mental retardation in girls, is associated with mutations of an X-linked gene encoding the transcriptional repressor protein MeCP2. Mecp2(1lox) mutant mice express no functional MeCP2 protein and exhibit behavioral abnormalities similar to those seen in RTT patients. Here we monitor the development of both whole brain and regional volumes between 21 and 42 days of age in this model of RTT using MRI. We see decreases in whole brain volumes in both male and female mutant mice. Cerebellar and ventricular volumes are also decreased in RTT males. Previous work has suggested that perinatal choline supplementation alleviates some of the behavioral deficits in both male and female Mecp2(1lox) mutant mice. Here we show that perinatal choline supplementation also positively affects whole brain volume in heterozygous females, and cerebellar volume in male RTT mice.
Ward
B C
BC
Neuroscience Program, Wellesley College, USA.
Agarwal
S
S
Wang
K
K
Berger-Sweeney
J
J
Kolodny
N H
NH
D000284:Administration, Oral; D000818:Animals; D001522:Behavior, Animal / Q000187:drug effects; D001835:Body Weight / Q000187:drug effects; D001921:Brain / Q000187:drug effects* / Q000473:pathology; D002794:Choline / Q000008:administration & dosage*; D004195:Disease Models, Animal; D005260:Female; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D051783:Methyl-CpG-Binding Protein 2 / Q000172:deficiency / Q000235:genetics; D051379:Mice; D008817:Mice, Mutant Strains; D018697:Nootropic Agents / Q000008:administration & dosage*; D015518:Rett Syndrome / Q000188:drug therapy* / Q000473:pathology
D051783:Methyl-CpG-Binding Protein 2; D018697:Nootropic Agents; D002794:Choline