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Absence of tubular structures and immunolabeling for von Willebrand factor
in the platelet alpha-granules from porcine von Willebrand disease
[published erratum appears in Blood 1987 Feb;69(2):707]
EM Cramer, JP Caen, L Drouet and J Breton-Gorius
The electron microscopic localization of von Willebrand factor (vWF) was
studied in platelets from normal and von Willebrand disease (vWD) pigs. In
normal pig platelets, immunolabeling for vWF was far more intense and
extensive than in human platelets and was either localized at one pole of
the alpha-granule or all along its periphery or long axis. As in human
platelets, this immunolabeling coincided with the presence of tubules about
200 nm in diameter. These structures were more numerous than in human
platelets, with up to 30 tubules per alpha- granule. They were easily
identified either in transverse sections, usually grouped in a less
electron-dense part of the matrix at one pole of the alpha-granule, or in
longitudinal sections parallel to the long axis of the elongated granules,
or coiled around the alpha-granule core. They closely resemble those
structures found in Weibel-Palade bodies. In platelets from pigs with
severe vWD, these structures were absent, as was the immunolabeling for
vWF; however, cytoplasmic microtubules were normally present in these
platelets. Thus, the granule-associated tubules can be distinguished from
the microtubules, which are larger in diameter (250 nm), are present in
both normal and vWD platelets, and do not stain for vWF. These results
strongly suggest that the tubular structures present in the alpha-granules
of normal porcine platelets correspond to the vWF molecule itself.
Volume 68,
Issue 3,
pp. 774-778,
09/01/1986
Copyright © 1986 by The American Society of Hematology
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