Survival motor neuron protein regulates stem cell division, proliferation, and differentiation in XXXX .

@entity1 @entity66 @entity73009 @entity179 @entity11280 @entity2057 @entity238
@entity2057 is a severe @entity66 that is caused by mutations in the @entity1 @entity11280 ( @entity11280 ) gene. @entity73009 protein is required for the assembly of small nuclear ribonucleoproteins and a dramatic reduction of the protein leads to cell death. It is currently unknown how the reduction of this ubiquitously essential protein can lead to tissue-specific abnormalities. In addition, it is still not known whether the disease is caused by developmental or @entity179 . Using the @entity238 system, we show that @entity73009 is enriched in postembryonic neuroblasts and forms a concentration gradient in the differentiating progeny. In addition to the developing @entity238 larval CNS, @entity238 larval and adult testes have a striking @entity73009 gradient. When @entity73009 is reduced in postembryonic neuroblasts using MARCM clonal analysis, cell proliferation and clone formation defects occur. These @entity73009 mutant neuroblasts fail to correctly localise Miranda and have reduced levels of snRNAs. When @entity73009 is removed, germline stem cells are lost more frequently. We also show that changes in @entity73009 levels can disrupt the correct timing of cell differentiation. We conclude that highly regulated @entity73009 levels are essential to drive timely cell proliferation and cell differentiation.
