Vj. Westlake et al., HEMATOPOIETIC-CELL TRANSPLANTATION IN FETAL LAMBS WITH CEROID-LIPOFUSCINOSIS, American journal of medical genetics, 57(2), 1995, pp. 365-368
Hematopoietic cells from the liver of normal 45-48-day-old fetal lambs
(Hb type AA) were transplanted intraperitoneally into 58-60-day-old r
ecipient fetuses (Hb type BB). The recipient fetuses resulted from mat
ing homozygous ceroid lipofuscinosis affected males with heterozygous,
phenotypically normal, females, The sex of the donor fetus was also r
ecorded, At age 2 1/2 months the recipient lambs with ceroid-lipofusci
nosis were diagnosed by histopathology of brain biopsies, Monitoring o
f blood and bone marrow cells showed that an average of 9% of blood ce
lls in ceroid-lipofuscinosis affected recipients were of donor origin,
No differences were evident in the clinical course of disease, brain
weight, or histopathology of organs between transplanted and non-trans
planted lambs with ceroid-lipofuscinosis. Under the conditions of this
experiment, transplantation of fetal hematopoietic cells was not bene
ficial. (C) 1995 Wiley-Liss, Inc.