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XB-ART-50201
PLoS Genet March 1, 2015; 11 (3): e1005018.

The ribosome biogenesis factor Nol11 is required for optimal rDNA transcription and craniofacial development in Xenopus.

Griffin JN , Sondalle SB , Del Viso F , Baserga SJ , Khokha MK .


Abstract
The production of ribosomes is ubiquitous and fundamental to life. As such, it is surprising that defects in ribosome biogenesis underlie a growing number of symptomatically distinct inherited disorders, collectively called ribosomopathies. We previously determined that the nucleolar protein, NOL11, is essential for optimal pre-rRNA transcription and processing in human tissue culture cells. However, the role of NOL11 in the development of a multicellular organism remains unknown. Here, we reveal a critical function for NOL11 in vertebrate ribosome biogenesis and craniofacial development. Nol11 is strongly expressed in the developing cranial neural crest (CNC) of both amphibians and mammals, and knockdown of Xenopus nol11 results in impaired pre-rRNA transcription and processing, increased apoptosis, and abnormal development of the craniofacial cartilages. Inhibition of p53 rescues this skeletal phenotype, but not the underlying ribosome biogenesis defect, demonstrating an evolutionarily conserved control mechanism through which ribosome-impaired craniofacial cells are removed. Excessive activation of this mechanism impairs craniofacial development. Together, our findings reveal a novel requirement for Nol11 in craniofacial development, present the first frog model of a ribosomopathy, and provide further insight into the clinically important relationship between specific ribosome biogenesis proteins and craniofacial cell survival.

PubMed ID: 25756904
PMC ID: PMC4354908
Article link: PLoS Genet
Grant support: [+]
Genes referenced: ap2a1 dlx5 dtl gnl3 gsc hoxb3 krt12.4 myod1 nkx2-5 nol11 not pax2 pitx2 rgn rrad slc5a1.1 snai2 sox3 sox9 tfap2a tp53 twist1
Morpholinos: nol11 MO1


Article Images: [+] show captions
References:
Aybar, 2002, Pubmed, Xenbase [+]


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