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Hematopoietic cell transplantation in fetal lambs with ceroid-lipofuscinosis.

作者信息

Westlake V J, Jolly R D, Jones B R, Mellor D J, Machon R, Zanjani E D, Krivit W

机构信息

Faculty of Veterinary Science, Massey University, Palmerston North, New Zealand.

出版信息

Am J Med Genet. 1995 Jun 5;57(2):365-8. doi: 10.1002/ajmg.1320570252.

DOI:10.1002/ajmg.1320570252
PMID:7668364
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.

摘要

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