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Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A by Kang, So-mi (author);Yoon, Min-Ho (author);Lee, Su-Jin (author);Ahn, Jinsook (author);Yi, Sang Ah (author);Nam, Ki Hong (author);Park, Soyoung (author);Woo, Tae-Gyun (author);Cho, Jung-Hyun (author);Lee, Jaecheol (author);Ha, Nam-Chul (author);Park, Bum-Joon (author) is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.

What is Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A about?

Werner syndrome (WRN) is a rare progressive genetic disorder, caused by functional defects in WRN protein and RecQ4L DNA helicase. Acceleration of the aging process is initiated at puberty and the expected life span is approximately the late 50 s. However, a __Wrn__-deficient mouse model does not show premature aging phenotypes or a short life span, implying that aging processes differ greatly between humans and mice. Gene expression analysis of WRN cells reveals very similar results to gene expression analysis of Hutchinson Gilford progeria syndrome (HGPS) cells, suggesting that these human progeroid syndromes share a common pathological mechanism. Here we show that WRN cells also express progerin, an abnormal variant of the lamin A protein. In addition, we reveal that duplicated sequences of human WRN (hWRN) from exon 9 to exon 10, which differ from the sequence of mouse WRN (mWRN), are a natural inhibitor of progerin. Overexpression of hWRN reduced progerin expression and aging features in HGPS cells. Furthermore, the elimination of progerin by siRNA or a progerin-inhibitor (SLC-D011 also called progerinin) can ameliorate senescence phenotypes in WRN fibroblasts and cardiomyocyt

Who reads Human WRN is an intrinsic inhibitor of progerin, abnormal splicing product of lamin A?

It is typically read by researchers, students, and practitioners in Biochemistry, Genetics and Molecular Biology.

Author
Kang, So-mi (author);Yoon, Min-Ho (author);Lee, Su-Jin (author);Ahn, Jinsook (author);Yi, Sang Ah (author);Nam, Ki Hong (author);Park, Soyoung (author);Woo, Tae-Gyun (author);Cho, Jung-Hyun (author);Lee, Jaecheol (author);Ha, Nam-Chul (author);Park, Bum-Joon (author)
Publisher
Springer Science and Business Media LLC
Published
2021
Language
EN
Field
Biochemistry, Genetics and Molecular Biology (Life Sciences)

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