Transcripts
DR. ROCK:
Most recently, Furlan has looked
at the congenital forms and been able to find
that in some small children who have recurrent
forms of TTP there is a deficiency in the
metalloprotease which reduces von Willebrand
factor.
Now, what this is all about is
you have normally secreted from the endothelium
von Willebrand factor -- the amounts
will increase when the endothelium is insulted,
such as by exposure to verotoxin. In fact, data
from my lab about 10 years ago showed when we
exposed normal cultured endothelial cells to
purified verotoxin, we got the release of von
Willebrand factor and unusually large forms of
von Willebrand factor from the endothelium.
