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B protein of factor XIII: differentiation between free B and complexed B
H Yorifuji, K Anderson, GW Lynch, L Van de Water and J McDonagh
Department of Pathology, Beth Israel Hospital, Boston, MA 02215.
Plasma factor XIII is a complex of A and B proteins noncovalently linked in
a tetramer, A2B2, Enzyme-linked immunosorbent assays (ELISA) were developed
to measure the separate factor XIII proteins and the complex. All of the A
protein in plasma is in the zymogen complex. The B assay measures the total
amount of B protein in plasma (both free B and complexed B). This was
confirmed by nondenaturing gel electrophoresis and immunoblotting, which
showed two bands for B in plasma with this antibody. Two assays were
developed to measure A2B2 complex specifically. One assay used a monoclonal
antibody to B to bind antigen and measured B protein in the zymogen complex
only and hence the concentration of the complex. The specificity of this
antibody was also shown by immunoblotting. In the second assay, the capture
antibody was to B and the tag antibody was to A. These two assays gave
identical results for the concentration of A2B2 (0.07 mumol/L, 21.6
micrograms/mL in normal plasma). Thus, for the first time, differentiation
and quantitation of free B and complexed B in plasma was possible. The
assays were used to measure factor XIII proteins in plasma from normal
controls, homozygous-deficient factor XIII patients, and their heterozygous
relatives. The normal concentration of A in plasma is 0.13 to 0.16 mumol/L
(approximately 11 micrograms/mL), all of which is in A2B2. The total B
concentration is 0.26 to 0.28 mumol/L (approximately 21 micrograms/mL),
half of which is complexed. The free B concentration is 0.13 mumol/L
(approximately 10 micrograms/mL). Homozygous-deficient patients have
essentially no A protein, but their free B concentration is 0.11 mumol/L.
Heterozygotes have decreased A2B2, but their free B is 0.11 mumol/L. These
results indicate that the concentration of free B is remarkably constant
and does not depend on the concentration of A2 or A2B2.
Volume 72,
Issue 5,
pp. 1645-1650,
11/01/1988
Copyright © 1988 by The American Society of Hematology
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