| 16897 | Q80Y17 | L2GL1_MOUSE | Lethal(2) giant larvae protein homolog 1 (LLGL) (Mgl-1) (Mlgl) | DISRUPTION PHENOTYPE | Mice exhibit disorganization and disruption of the apical junctional complex, resulting in hyper-proliferation of neuroblasts and brain dysplasia. Loss of Lgl1 in mice results in formation of neuroepithelial rosette-like structures, similar to the neuroblastic rosettes in human primitive neuroectodermal tumors. The newborn Lgl1(-/-) pups develop severe hydrocephalus and die neonatally. Due to the loss of mitotic spindle orientation, a large proportion of Lgl1(-/-) neural progenitor cells fails to exit the cell cycle and differentiate, and, instead, continues to proliferate and dies by apoptosis. Dividing Lgl1(-/-) cells are unable to asymmetrically localize the Notch inhibitor Numb, and the resulting failure of asymmetric cell divisions may be responsible for the hyperproliferation and the lack of differentiation. {ECO:0000269|PubMed:15037549}. |