Figure 1.
ADAMTS13 activity levels in the plasma samples of the patient and of her parents. (A) An immunoblot depicts the generation of specific VWF fragments, the homodimers of the 176-kDa ([176 kDa]2) and the 140-kDa ([140 kDa]2) fragments,15 from the VWF substrate by ADAMTS13. NP indicates normal control plasma; I-1, the father; I-2, the mother; and II-1, the patient. Each sample was analyzed in the absence (–) or presence (+) of EDTA (ethylenediaminetetraacetic acid). (B) The pedigree depicts the ADAMTS13 activity levels of the father (I-1), the mother (I-2), and the patient (II-1). The values, expressed in U/mL (1 U/mL being the activity in the normal control plasma), are the means of 3 separate assays. The normal range of the assay is 0.79 to 1.27 U/mL.