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Modulation of LMNA splicing as a strategy to treat prelamin A diseases.
Lee JM, Nobumori C, Tu Y, Choi C, Yang SH, Jung HJ, Vickers TA, Rigo F, Bennett CF, Young SG, Fong LG. Lee JM, et al. Among authors: nobumori c. J Clin Invest. 2016 Apr 1;126(4):1592-602. doi: 10.1172/JCI85908. Epub 2016 Mar 21. J Clin Invest. 2016. PMID: 26999604 Free PMC article.
Mice that express farnesylated versions of prelamin A in neurons develop achalasia.
Yang SH, Procaccia S, Jung HJ, Nobumori C, Tatar A, Tu Y, Bayguinov YR, Hwang SJ, Tran D, Ward SM, Fong LG, Young SG. Yang SH, et al. Among authors: nobumori c. Hum Mol Genet. 2015 May 15;24(10):2826-40. doi: 10.1093/hmg/ddv043. Epub 2015 Feb 4. Hum Mol Genet. 2015. PMID: 25652409 Free PMC article.
Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice.
Coffinier C, Jung HJ, Li Z, Nobumori C, Yun UJ, Farber EA, Davies BS, Weinstein MM, Yang SH, Lammerding J, Farahani JN, Bentolila LA, Fong LG, Young SG. Coffinier C, et al. Among authors: nobumori c. J Biol Chem. 2010 Jul 2;285(27):20818-26. doi: 10.1074/jbc.M110.128835. Epub 2010 May 3. J Biol Chem. 2010. PMID: 20439468 Free PMC article.
An absence of nuclear lamins in keratinocytes leads to ichthyosis, defective epidermal barrier function, and intrusion of nuclear membranes and endoplasmic reticulum into the nuclear chromatin.
Jung HJ, Tatar A, Tu Y, Nobumori C, Yang SH, Goulbourne CN, Herrmann H, Fong LG, Young SG. Jung HJ, et al. Among authors: nobumori c. Mol Cell Biol. 2014 Dec;34(24):4534-44. doi: 10.1128/MCB.00997-14. Epub 2014 Oct 13. Mol Cell Biol. 2014. PMID: 25312645 Free PMC article.
13 results