Phenotype Image Detail

Image

Caption

Hydrocephalus and enlarged pS6+ cortical cells in Tsc1^tm1Djk/Tsc1^tm1Djk mice injected with AAV1-CBA-Cre vector. Tsc1^tm1Djk/Tsc1^tm1Djk Gt(ROSA)26Sor^tm1Sho/Gt(ROSA)26Sor⁺ pups were injected ICV at P0 with either an AAV1-CBA-GFP (N = 6) or AAV1-CBA-Cre (N = 10) vector at a concentration of 2x10¹⁰ g.c per ventricle. One month later pups which had received AAV1-CBA-Cre virus developed tremor (N = 8) and were found to have hydrocephalus by pathologic analysis (N = 2). (A) Representative images of brain (upper panels) and cerebral cortex (lower panels) showing in AAV1-CBA-Cre injected animals severe hydrocephalus, large portion of enlarged cortical cells with strong pS6-positivity, and condensed cortical thickness due to swelling of ventricles. Scale bars = 1 mm, upper; 100 um, lower. (B) Representative pS6 staining in different brain regions. Enlarged pS6+ cells were ectopically present in striatum oriens of hippocampus (arrowhead) due to migration defect of Tsc1-null cells. Some pS6+ Purkinje cells in the cerebellum as well as neurons in the caudate were notably enlarged (arrowheads). Neural cells in deep nuclei such as the brain stem showed similar distribution of pS6 positivity in Cre and GFP injected mice. Scale bars = 50 um.

Copyright

This image is from Prabhakar S, PLoS One 2013;8(5):e64224, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:221022

Associated Alleles

Symbol	Name
Gt(ROSA)26Sor^tm1Sho	gene trap ROSA 26, Philippe Soriano; targeted mutation 1, Stuart Orkin
Tsc1^tm1Djk	TSC complex subunit 1; targeted mutation 1, David J Kwiatkowski

Associated Genotypes

Allelic Composition	Genetic Background
Tsc1^tm1Djk/Tsc1^tm1Djk Gt(ROSA)26Sor^tm1Sho/Gt(ROSA)26Sor^+	involves: 129S4/SvJae * 129S4/SvJaeSor