Most strongly impaired in kon (a 3.5 fold decrease) was the infantile Refsum disease mutant Pex5p(C)S600W [7], a mutation in the 7C-loop (and therefore distant from the ancillary interface) we have previously found not to bind to mSCP2 in ITC experiments and to impair import of SCP2 and catalase in vivo [4]. Here, SCP2 is linked to Refsum disease.