Abstract
The nucleoli are membrane-less nuclear substructures that govern ribosome biogenesis and participate in multiple other cellular processes such as cell cycle progression, stress sensing, and DNA damage response. The proper functioning of these organelles is ensured by specific proteins that maintain nucleolar structure and mediate key nucleolar activities. Among all nucleolar proteins, treacle encoded by TCOF1 gene emerges as one of the most crucial regulators of cellular processes. TCOF1 was initially discovered as a gene involved in the Treacher Collins syndrome, a rare genetic disorder characterized by severe craniofacial deformations. Later studies revealed that treacle regulates ribosome biogenesis, mitosis, proliferation, DNA damage response, and apoptosis. Importantly, several reports indicate that treacle is also involved in cancer development, progression, and response to therapies, and may contribute to other pathologies such as Hirschsprung disease. In this manuscript, we comprehensively review the structure, function, and the regulation of TCOF1/treacle in physiological and pathological processes.
Highlights
Many of the crucial cellular processes that play an important role in proper functioning of the body occur in the nucleoli—small nuclear substructures devoid of a surrounding membrane [1,2,3]
When we consider the crucial roles of treacle in biogenesis of ribosomes, DNA damage response, and mitotic regulation, it is not surprising that TCOF1 dysfunction can lead to developmental disorders and can affect the functioning of mature cells
Since ribosome biogenesis can be regulated by non-coding RNAs [103], it may suggest that TCOF1 could be a target of such regulation
Summary
Many of the crucial cellular processes that play an important role in proper functioning of the body occur in the nucleoli—small nuclear substructures devoid of a surrounding membrane [1,2,3]. Apart from its primary function, the biogenesis of ribosomes, the nucleolus is involved in cell cycle progression, stress sensing, and DNA damage response All these nucleolar activities are mediated by multiple proteins that ensure proper nucleolar structure and function [1,7,8,9,10]. Recent studies provide evidence that treacle regulates key cellular processes affecting development of facial skeleton, and contributing to other pathological processes including cancer. In this manuscript, we comprehensively review the structure, function, and regulation of TCOF1/treacle both in physiological and pathological processes
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