ELP1 and Fabry disease: To evaluate whether the axonal transport defects we detected in CKOTyrp2 FD DRGs are due to the effect of IKAP on acetylation of α-tubulin, we used immunoblot assays to quantify acetylated α-tubulin in FD CKOTyrp2 mouse DRGs and brains, in fibroblasts derived from FD patients, and in HEK 293nt cells in which IKAP levels were reduced by stable expression of a shRNA targeted to the IKBKAP mRNA (shIKAP) (Fig 4A–4D; *p<0.05).