By John B. Graham, Emily S. Barrow, Howard M. Reisner (auth.), Harry Harris, Kurt Hirschhorn (eds.)
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Extra info for Advances in Human Genetics
There may be some duplication of patients among reports. Classification by original authors was used when available. When unavailable, values ",;5% judged as CRM -, ~60% judged as CRM + . This classification was used to make inhibitor neutralization results comparable to radioimmunoassay or electroimmunoassay (Laurel\) results. C Summary of older data; classification of Roberts used. d Inhibitor neutralization assay data. b phasize the need for caution in comparisons made using different antibodies.
The smallest multi mer appears to represent a disulfide-bonded tetramer of the basic 200,000 subunit (see Fig. 4). 4 X 106 , . • , 12 X 106 (Hoyer, 1981). , 1978). The multimers can be dissociated into subunits of My ~200,000 by reduction with dithiothreitol or l3-mercaptoethanol but not 6 M guanidine or 1% SDS alone (Hoyer, 1981). Biochemical characterization of highly purified human VIlIR:WF has demonstrated the presence of carbohydrate (5-6%), with galactose, glucosamine, and sialic acid being specific components (G.
Factor VII is exceptional in that activation proceeds without loss of mass, only the amino-terminal cleavage occurring. The activation peptide region of activated F. , 1979; Jackson and Nemerson, 1979; Jackson, 1981; Zur and Nemerson, 1981). The role of the released activation peptides, if any, is unknown. The Catalytic Domain The carboxy-terminal catalytic region of the vitamin K-dependent proteases consists of about 250 residues with strong structural homology to the pancreatic proteases. Forty to fifty percent of the residues are identical in pairwise comparisons between Fs.