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Journal of Zhejiang University SCIENCE B 1998 Vol.-1 No.-1 P.

http://doi.org/10.1631/jzus.B2300904


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’ more

Corresponding email(s):   daijiewen@163.com, 839593414@qq.com

Key Words:  Ribosome, Phase separation, Ribosomopathy, Tissue specificity


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, 1998, -1(-1): .

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author="Zhiyuan PAN, Guofen LIN, Hao LIU, Guozhi LI, Xiaoyi ZHANG, Jiewen DAI",
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year="1998",
publisher="Zhejiang University Press & Springer",
doi="10.1631/jzus.B2300904"
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%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
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%D 1998
%I Zhejiang University Press & Springer
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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
VL - -1
IS - -1
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PB - Zhejiang University Press & Springer
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DOI - 10.1631/jzus.B2300904


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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