Domain structure of Nebulin and antibodies available antibodies from Myomedix:

Domain Structure of the nebulin protein. About 97% of nebulin´s 800 kDa sized polypeptide chain is composed of 35-residue helical repeats. The antibodies available from Myomedix recognize unique epitopes located at nebulin´s N-terminal end (NEB-NH2, located at the pointed thin filament end), or at its C-terminal end (NEB-COOH, located at its Z-line end). The nebulin antibodies are useful to detect nebulin deficiency in nemaline myopathy disease (Ottenheijm et al., 2012).