BSND and Bloom syndrome: Importantly, a molecule able to increase protein stability and prevent ER degradation, such as 17-allylamino-17-demethoxygeldanamycin (17-AAG), an Hsp90 inhibitor, was reported to mitigate the BS symptoms in barttin R8L knock-in mice by likely enhancing the plasma membrane expression of ClC-K1/mutant barttin channels (Nomura et al., 2013).