Similarly, the structural analysis of two other mutations in the β1 propeller domain of LRP4 identified as responsible for Cenani–lenz syndrome (Asp529Asn, Arg545Trp), revealed that these exposed residues are also not directly involved in the agrin/LRP4 interaction, but such substitutions could affect the global stability of LRP4 and can consequently destabilize the agrin/LRP4 complex (data not shown). Here, AGRN is linked to Cenani-Lenz syndrome.