Haploinsufficiency of ribosomal proteins and p53 activation in anemia: Diamond-Blackfan anemia and the 5q-syndrome

J Boultwood, A Pellagatti, JS Wainscoat - Advances in biological regulation, 2012 - Elsevier
The p53 pathway responds to a wide variety of cellular stress signals including impaired
ribosomal biogenesis. It is now well recognized that in conditions of 'ribosomal stress'
activation of p53 occurs, promoting the transcription of its many target genes and resulting in
p53-dependent cell cycle arrest, senescence, or apoptosis (Riley et al., 2008). There is
increasing evidence that p53 activation is a common response to reduced levels of
ribosomal proteins in a number of human disorders of defective ribosomal biogenesis, most …