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. 2003 Apr 14;72(5):1300–1307. doi: 10.1086/375116

Figure 4.

Figure 4

Immunological detection of MHBD in cultured skin fibroblasts from patients with MHBD deficiency. For immunoblot analysis, 50 μg protein of cultured skin fibroblasts from control subjects and patients with MHBD deficiency was applied on a 10% polyacrylamide gel and separated, as described elsewhere (Laemmli 1970). After electrophoresis, proteins were transferred to a nitrocellulose membrane by semidry blotting, probed with the polyclonal antibody raised against recombinant human MHBD, and developed (Wanders et al. 1995). After staining of the blot with NBT/BCIP, the result was digitalized by use of a desktop scanner and the amount of MHBD protein quantified, using the National Insitutes of Health Image 1.62 software program. CRIM = cross reactive immunological material. Control-mean value of three control subjects represented as 100% (±SD); 1 = patient 1; 2 = patient 2; 3 = patient 3, and 5 = patient 5 (see table 1).