For example, both KBTBD1 (Klhl31) and KBTBD10 (Klhl41) depletion cause nemaline myopathy, although the mechanisms are different: while KLHL31 targets Filamin C, a muscle-specific actin-crosslinking protein, for ubiquitination and degradation [23], KLHL41 is poly-ubiquitinated and prevents aggregation and degradation of nebulin, an essential component of the sarcomere. Here, NEB is linked to nemaline myopathy.