Table 1. Ependymal cilia defects are proportionate to the catalytic activities of various mutant forms of SHP2.
Mice | Catalytic activity of mutant SHP2 | Ependymal cilia |
---|---|---|
Ptpn11E76K/+/Nestin-Cre+ | Substantially enhanced | Severely abnormal |
Ptpn11D61G/+ (NS mice) | Enhanced | Abnormal in some areas * |
Ptpn11Y279C/+ (NSML mice) | No catalytic activity | Normal |
Ependymal cilia of the third ventricle, but not the lateral ventricles, were abnormal.