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Hematopoietic cell transplantation in fetal lambs with ceroid-lipofuscinosis.

Abstract
Hematopoietic cells from the liver of normal 45-48-day-old fetal lambs (Hb type AA) were transplanted intraperitoneally into 58-60-day-old recipient fetuses (Hb type BB). The recipient fetuses resulted from mating homozygous ceroid-lipofuscinosis affected males with heterozygous, phenotypically normal, females. The sex of the donor fetus was also recorded. At age 2 1/2 months the recipient lambs with ceroid-lipofuscinosis were diagnosed by histopathology of brain biopsies. Monitoring of blood and bone marrow cells showed that an average of 9% of blood cells 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-transplanted lambs with ceroid-lipofuscinosis. Under the conditions of this experiment, transplantation of fetal hematopoietic cells was not beneficial.
AuthorsV J Westlake, R D Jolly, B R Jones, D J Mellor, R Machon, E D Zanjani, W Krivit
JournalAmerican journal of medical genetics (Am J Med Genet) Vol. 57 Issue 2 Pg. 365-8 (Jun 05 1995) ISSN: 0148-7299 [Print] United States
PMID7668364 (Publication Type: Journal Article)
Topics
  • Animals
  • Atrophy
  • Brain (pathology)
  • Crosses, Genetic
  • Female
  • Fetal Tissue Transplantation
  • Fetus
  • Hematopoietic Stem Cell Transplantation
  • Kidney (pathology)
  • Liver (pathology)
  • Male
  • Neuronal Ceroid-Lipofuscinoses (pathology, therapy, veterinary)
  • Pregnancy
  • Retina (pathology)
  • Sex Characteristics
  • Sheep
  • Sheep Diseases

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