Table 1.

ECM related cellular adaptive responses in diseases.

Gene/gene product disrupted	Cellular adaptive response demonstrated	Reference
mdx/utr, mdx/itga7, or utr/itga7 double knock out mice	Compensation between Dystrophin, Utrophin, and Integrin alpha7	Guo et al., 2006; Rooney et al., 2006; Deconinck et al., 1997; Grady et al., 1997; Welser et al., 2009
Dystroglycan/Integrin alpha7, Dystroglycan/Integrin-linked kinase, or Dystrophin/Integrin-linked kinase double knockdown in zebrafish	Compensation between Dystroglycan or Dystrophin and Integrin alpha7 and/or Integrin linked kinase	Goody et al., 2012; Sztal et al., 2012
mdx mice	Sarcospan overexpression sufficient to increase DGC, UGC, and Integrin alph7beta1 sarcolemma expression	Marshall and Crosbie-Watson, 2013
Human DMD	Increased Utrophin is beneficial	Fairclough et al., 2013
lama2	Partial compensation by increased laminin alpha4	Kortesmaa et al., 2010; Talts et al., 2010
lama2, mdx, or itga7 mutant mice	Laminin-111 protein therapy beneficial	Rooney et al., 2009a; 2009b; 2012
lamb2 mutant zebrafish	Fibers form and attach to ectopic BMs	Jacoby et al., 2009
Integrin alpha7 or Dystroglycan deficient zebrafish	Exogenous NAD+ supplementation enhances basement membrane	Goody et al., 2012
dmd mutant zebrafish	Phosphodiesterase inhibitors, upregulation of heme oxygenase 1, or selective serotonin reuptake inhibitors beneficial	Kawahara et al., 2011; 2014; Waugh et al., 2014
itga7 mutant mice	Integrin alpha5 and Fn are upregulated	Nawrotzki et al., 2003
lama2 mutant mice	Fn is upregulated	Mehuron et al., 2014
Decorin/Biglycan double knock out mice	Compensation between Decorin and Biglycan in collagen fibrillogenesis in bone and cornea	Young et al., 2002; Zhang et al., 2009
col4a3 mutant mice	Upregulation of col4a1, col4a2, lama1, and lama5	Abrahamson et al., 2007