... tissue-non-specific alkaline phosphatase with an Arg54 fi Cys substitution and a counterpart with an Asp277 fi Ala substitution found in a compound heterozygote associated with severe hypophosphatasia ... the final concentrations were (lg ⁄ mL): lanes and 6, 0; lanes and 7, 5; lanes and 8, 10; lanes and 9, 20; lanes and 10, 50 For proteinase K, the final concentrations were (lg ⁄ mL): lanes and ... lanes and 6, 0; lanes and 7, 0.5; lanes and 8, 1.0; lanes and 9, 5.0; lanes and 10, 10 Lanes 1–5 and lanes 6–10 were analyzed by SDS ⁄ PAGE in the presence or absence of 2-mercaptoethanol, respectively...