1998; Brini and Carafoli 2009). Ile 235 and Glu 982 are highly conserved in SERCA1 throughout different species (data not shown). Ile 235 is located in the A domain where the majority of ATP2A1 mutations associated with Brody myopathy have been found (Fig. S1). Ile 235 lies at the beginning of the α3 helix of the A domain (Fig.2), where a change from a nonpolar isoleucine to a polar asparagine may have a destabilizing effect on SERCA1 function. Glu 982 lies within the last TM domain (TM10) of the protein (Fig.2). The gene discussed is ATP2A1; the disease is Brody myopathy.