Another studies point out the crucial role of ER stress in diseases caused by mutations in collagen type VI genes that lead to the mild to severe phenotype of myopathies, including Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy (BM) (De Palma et al. 2014), or mutations in collagen type IV genes (COL4A3/COL4A4/COL4A5) that contribute to the pathogenesis of thin-basement-membrane nephropathy (TBMN) and Alport syndrome (AS) (Pieri et al. 2014) and cataract development (Firtina et al. 2009). Here, COL4A3 is linked to Ullrich congenital muscular dystrophy.