Commentary: SU9516 Increases α7β1 Integrin and Ameliorates Disease Progression in the Mdx Mouse Model of Duchenne Muscular Dystrophy

Overview

Journal J Rare Dis Res Treat

Date 2019 Mar 19

PMID 30882096

Authors

Apurva Sarathy

Andreia M Nunes

Tatiana M Fontelonga

Tracy Y Ogata

Dean J Burkin

Affiliations

Soon will be listed here.

References

Burkin D, Kaufman S . The alpha7beta1 integrin in muscle development and disease. Cell Tissue Res. 1999; 296(1):183-90. DOI: 10.1007/s004410051279. View

Burkin D, Wallace G, Nicol K, Kaufman D, Kaufman S . Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic mice. J Cell Biol. 2001; 152(6):1207-18. PMC: 2199213. DOI: 10.1083/jcb.152.6.1207. View

Lane M, Yu B, Rice A, Lipson K, Liang C, Sun L . A novel cdk2-selective inhibitor, SU9516, induces apoptosis in colon carcinoma cells. Cancer Res. 2001; 61(16):6170-7. View

Yu B, Lane M, Wadler S . SU9516, a cyclin-dependent kinase 2 inhibitor, promotes accumulation of high molecular weight E2F complexes in human colon carcinoma cells. Biochem Pharmacol. 2002; 64(7):1091-100. DOI: 10.1016/s0006-2952(02)01264-9. View

Burkin D, Wallace G, Milner D, Chaney E, Mulligan J, Kaufman S . Transgenic expression of {alpha}7{beta}1 integrin maintains muscle integrity, increases regenerative capacity, promotes hypertrophy, and reduces cardiomyopathy in dystrophic mice. Am J Pathol. 2005; 166(1):253-63. PMC: 1602287. DOI: 10.1016/s0002-9440(10)62249-3. View

Gao N, Kramer L, Rahmani M, Dent P, Grant S . The three-substituted indolinone cyclin-dependent kinase 2 inhibitor 3-[1-(3H-imidazol-4-yl)-meth-(Z)-ylidene]-5-methoxy-1,3-dihydro-indol-2-one (SU9516) kills human leukemia cells via down-regulation of Mcl-1 through a transcriptional mechanism. Mol Pharmacol. 2006; 70(2):645-55. DOI: 10.1124/mol.106.024505. View

Rooney J, Welser J, Dechert M, Flintoff-Dye N, Kaufman S, Burkin D . Severe muscular dystrophy in mice that lack dystrophin and alpha7 integrin. J Cell Sci. 2006; 119(Pt 11):2185-95. DOI: 10.1242/jcs.02952. View

Ervasti J . Dystrophin, its interactions with other proteins, and implications for muscular dystrophy. Biochim Biophys Acta. 2006; 1772(2):108-17. DOI: 10.1016/j.bbadis.2006.05.010. View

Liu J, Burkin D, Kaufman S . Increasing alpha 7 beta 1-integrin promotes muscle cell proliferation, adhesion, and resistance to apoptosis without changing gene expression. Am J Physiol Cell Physiol. 2007; 294(2):C627-40. PMC: 2593740. DOI: 10.1152/ajpcell.00329.2007. View

10.

Stedman H, Sweeney H, Shrager J, MAGUIRE H, Panettieri R, PETROF B . The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy. Nature. 1991; 352(6335):536-9. DOI: 10.1038/352536a0. View

11.

Schara U, Mortier , Mortier W . Long-Term Steroid Therapy in Duchenne Muscular Dystrophy-Positive Results versus Side Effects. J Clin Neuromuscul Dis. 2008; 2(4):179-83. DOI: 10.1097/00131402-200106000-00002. View

12.

Welser J, Rooney J, Cohen N, Gurpur P, Singer C, Evans R . Myotendinous junction defects and reduced force transmission in mice that lack alpha7 integrin and utrophin. Am J Pathol. 2009; 175(4):1545-54. PMC: 2751551. DOI: 10.2353/ajpath.2009.090052. View

13.

Lu A, Proto J, Guo L, Tang Y, Lavasani M, Tilstra J . NF-κB negatively impacts the myogenic potential of muscle-derived stem cells. Mol Ther. 2011; 20(3):661-8. PMC: 3293604. DOI: 10.1038/mt.2011.261. View

14.

Mendell J, Shilling C, Leslie N, Flanigan K, Al-Dahhak R, Gastier-Foster J . Evidence-based path to newborn screening for Duchenne muscular dystrophy. Ann Neurol. 2012; 71(3):304-13. DOI: 10.1002/ana.23528. View

15.

McAdam L, Mayo A, Alman B, Biggar W . The Canadian experience with long-term deflazacort treatment in Duchenne muscular dystrophy. Acta Myol. 2012; 31(1):16-20. PMC: 3440807. View

16.

Gordon B, Delgado Diaz D, Kostek M . Resveratrol decreases inflammation and increases utrophin gene expression in the mdx mouse model of Duchenne muscular dystrophy. Clin Nutr. 2012; 32(1):104-11. DOI: 10.1016/j.clnu.2012.06.003. View

17.

Wuebbles R, Sarathy A, Kornegay J, Burkin D . Levels of α7 integrin and laminin-α2 are increased following prednisone treatment in the mdx mouse and GRMD dog models of Duchenne muscular dystrophy. Dis Model Mech. 2013; 6(5):1175-84. PMC: 3759337. DOI: 10.1242/dmm.012211. View

18.

Mendell J, Rodino-Klapac L, Sahenk Z, Roush K, Bird L, Lowes L . Eteplirsen for the treatment of Duchenne muscular dystrophy. Ann Neurol. 2013; 74(5):637-47. DOI: 10.1002/ana.23982. View

19.

Young C, Pyle A . Exon Skipping Therapy. Cell. 2016; 167(5):1144. DOI: 10.1016/j.cell.2016.10.050. View

20.

Sarathy A, Wuebbles R, Fontelonga T, Tarchione A, Mathews Griner L, Heredia D . SU9516 Increases α7β1 Integrin and Ameliorates Disease Progression in the mdx Mouse Model of Duchenne Muscular Dystrophy. Mol Ther. 2017; 25(6):1395-1407. PMC: 5474963. DOI: 10.1016/j.ymthe.2017.03.022. View