The nuclear envelope protein Net39 is essential for muscle nuclear integrity and chromatin organization

Andres Ramirez-Martinez, Yichi Zhang, Kenian Chen, Jiwoong Kim, Bercin K. Cenik, John R. McAnally, Chunyu Cai, John M. Shelton, Jian Huang, Ana Brennan, Bret M. Evers, Pradeep P. A. Mammen, Lin Xu, Rhonda Bassel-Duby, Ning Liu () and Eric N. Olson ()
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Andres Ramirez-Martinez: University of Texas Southwestern Medical Center
Yichi Zhang: University of Texas Southwestern Medical Center
Kenian Chen: Quantitative Biomedical Research Center University of Texas Southwestern Medical Center
Jiwoong Kim: Quantitative Biomedical Research Center University of Texas Southwestern Medical Center
Bercin K. Cenik: University of Texas Southwestern Medical Center
John R. McAnally: University of Texas Southwestern Medical Center
Chunyu Cai: University of Texas Southwestern Medical Center
John M. Shelton: University of Texas Southwestern Medical Center
Jian Huang: University of Texas Southwestern Medical Center
Ana Brennan: University of Texas Southwestern Medical Center
Bret M. Evers: University of Texas Southwestern Medical Center
Pradeep P. A. Mammen: University of Texas Southwestern Medical Center
Lin Xu: Quantitative Biomedical Research Center University of Texas Southwestern Medical Center
Rhonda Bassel-Duby: University of Texas Southwestern Medical Center
Ning Liu: University of Texas Southwestern Medical Center
Eric N. Olson: University of Texas Southwestern Medical Center

Nature Communications, 2021, vol. 12, issue 1, 1-12

Abstract: Abstract Lamins and transmembrane proteins within the nuclear envelope regulate nuclear structure and chromatin organization. Nuclear envelope transmembrane protein 39 (Net39) is a muscle nuclear envelope protein whose functions in vivo have not been explored. We show that mice lacking Net39 succumb to severe myopathy and juvenile lethality, with concomitant disruption in nuclear integrity, chromatin accessibility, gene expression, and metabolism. These abnormalities resemble those of Emery–Dreifuss muscular dystrophy (EDMD), caused by mutations in A-type lamins (LMNA) and other genes, like Emerin (EMD). We observe that Net39 is downregulated in EDMD patients, implicating Net39 in the pathogenesis of this disorder. Our findings highlight the role of Net39 at the nuclear envelope in maintaining muscle chromatin organization, gene expression and function, and its potential contribution to the molecular etiology of EDMD.

Date: 2021
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DOI: 10.1038/s41467-021-20987-x

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