JZUS - Journal of Zhejiang University SCIENCE

Journal of Zhejiang University SCIENCE B

Accepted manuscript available online (unedited version)

Mechanisms of ribosomopathy and phase separation-related ribosomopathy

Author(s): Zhiyuan PAN, Guofen LIN, Hao LIU, Guozhi LI, Xiaoyi ZHANG, Jiewen DAI
Affiliation(s): Department of Oral and Cranio-Maxillofacial Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine; more
Corresponding email(s): daijiewen@163.com, 839593414@qq.com
Key Words: Ribosome; Phase separation; Ribosomopathy; Tissue specificity

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Zhiyuan PAN, Guofen LIN, Hao LIU, Guozhi LI, Xiaoyi ZHANG, Jiewen DAI. Mechanisms of ribosomopathy and phase separation-related ribosomopathy[J]. Journal of Zhejiang University Science B,in press.Frontiers of Information Technology & Electronic Engineering,in press.https://doi.org/10.1631/jzus.B2300904

@article{title="Mechanisms of ribosomopathy and phase separation-related ribosomopathy",
author="Zhiyuan PAN, Guofen LIN, Hao LIU, Guozhi LI, Xiaoyi ZHANG, Jiewen DAI",
journal="Journal of Zhejiang University Science B",
year="in press",
publisher="Zhejiang University Press & Springer",
doi="https://doi.org/10.1631/jzus.B2300904"
}

%0 Journal Article
%T Mechanisms of ribosomopathy and phase separation-related ribosomopathy
%A Zhiyuan PAN
%A Guofen LIN
%A Hao LIU
%A Guozhi LI
%A Xiaoyi ZHANG
%A Jiewen DAI
%J Journal of Zhejiang University SCIENCE B
%P
%@ 1673-1581
%D in press
%I Zhejiang University Press & Springer
doi="https://doi.org/10.1631/jzus.B2300904"

TY - JOUR
T1 - Mechanisms of ribosomopathy and phase separation-related ribosomopathy
A1 - Zhiyuan PAN
A1 - Guofen LIN
A1 - Hao LIU
A1 - Guozhi LI
A1 - Xiaoyi ZHANG
A1 - Jiewen DAI
J0 - Journal of Zhejiang University Science B
SP -
EP -
%@ 1673-1581
Y1 - in press
PB - Zhejiang University Press & Springer
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doi="https://doi.org/10.1631/jzus.B2300904"

Abstract
Chinese Summary
Academic Network
Reviewer Comment

Abstract: Ribosome is an intracellular ribonucleoprotein particle that serves as the site of protein biosynthesis. Ribosomal dysfunction caused by mutations in genes encoding ribosomal proteins (rPs) and ribosomal biogenesis factors (RBFs) can lead to a spectrum of diseases, collectively known as ribosomopathy. Phase separation is a thermodynamic process that produces multiple phases from a homogeneous mixture. The formation of membrane-less organelles and intracellular structures, including ribosomes and nucleoli, cannot occur without the involvement of phase separation. Here, the ribosome structure, biogenesis, and their relationship to ribosomopathy are systematically reviewed. The tissue specificity of ribosomopathy and the role of phase separation in ribosomopathy are particularly discussed, which may offer some clues for understanding the mechanisms of ribosomopathy. Then, some new ideas for the prevention, diagnosis, and treatment of ribosomopathy are provided.

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