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A GT in the Rest gene ( Rest GT ) causes REST deficiency and embryonic lethality.
Although REST is a critical transcriptional regulator in neurogenesis, the lack of an obvious neuronal phenotype in the REST knockout model prompted the author "re-open" the case by generating a novel mouse model of REST deficiency.
However, the causal role of REST deficiency in cancer progression is not yet clearly demonstrated.
Using this line, we generate mice with a REST deficiency in nestin-positive neural progenitors, prior to the time when REST is dismissed normally from chromatin during neurogenesis.
In fact, the expression of laminin subunits correlates with severity of phenotypic effects of Rest deficiency.
Furthermore, in contrast to recent observations [9], we also found that Rest deficiency (50% to 100%) did not affect the pluripotency of ES cells as adjudged by the expression levels of Oct4 and Nanog (Fig. S1E).
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10.7554/eLife.09584.004 Figure 2. A conditional Rest GT allele results in REST-deficiency in Nestin+ progenitors.
Address sleep deficiency.
This information is now included in the third paragraph of the subsection "Conditional REST gene deficiency in neural progenitors" and the legend to Figure 2F.
In this study, the DMD iPSC-CM model manifests cardiac abnormalities that are also seen in DMD patients, such as dystrophin deficiency, resting Ca2+ overload and increased apoptosis.
With a view to the development of (skin-) malignancies, we also have to address the question whether a decrease in DNA repair capacity of a cell population rests on complete repair deficiency of single cells (while others remained unaffected) or on a partial decrease of DNA repair capacity in all cells.
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