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Long-term enzyme replacement therapy in beta-glucuronidase--deficient mice by allogeneic bone marrow transplantation

Journal Article · · J. Lab. Clin. Med.; (United States)
OSTI ID:7055241
; ; ; ;
Enzyme replacement therapy was successfully accomplished in beta-Glu-deficient C3H/HeJ mice after transplantation of BM cells obtained from normal BALB/c donors. Marrow recipients were prepared for transplantation by fractionated TLI. Enzyme activity increased from 20.5 +/- 7.0 nmol/mg of protein per hour to 180 +/- 30.2 in the liver (p less than 0.001) and from 8.2 +/- 2.0 to 17.5 +/- 5.0 nmol/ml/hr in the plasma (p less than 0.05) at 50 days after marrow infusion. Normal enzyme activity was maintained in treated mice for at least 100 days after marrow transplantation, as documented by repeated liver biopsies and examination of plasma samples. The marrow donors and the recipients were fully histoincompatible. Both immunologic rejection of the marrow allograft and GVHD were prevented by the prior conditioning of the recipients with TLI, resulting in bilateral transplantation tolerance of host vs. graft and graft vs. host. The data suggest that allogeneic BM transplantation may provide a possible therapeutic approach for certain enzyme deficiency syndromes.
Research Organization:
Hadassah University Hospital, Jerusalem, Israel
OSTI ID:
7055241
Journal Information:
J. Lab. Clin. Med.; (United States), Journal Name: J. Lab. Clin. Med.; (United States) Vol. 99:6; ISSN JLCMA
Country of Publication:
United States
Language:
English

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Related Subjects

550603* -- Medicine-- External Radiation in Therapy-- (1980-)
560152 -- Radiation Effects on Animals-- Animals
62 RADIOLOGY AND NUCLEAR MEDICINE
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
ANIMAL CELLS
ANIMALS
BIOLOGICAL EFFECTS
BIOLOGICAL RADIATION EFFECTS
BONE MARROW CELLS
CONNECTIVE TISSUE CELLS
DISEASES
ENZYME ACTIVITY
EXTERNAL IRRADIATION
FRACTIONATED IRRADIATION
GRAFT-HOST REACTION
IMMUNOSUPPRESSION
IRRADIATION
LYMPHATIC SYSTEM
MAMMALS
MEDICINE
METABOLIC DISEASES
MICE
NUCLEAR MEDICINE
RADIATION EFFECTS
RADIOLOGY
RADIOTHERAPY
RODENTS
SOMATIC CELLS
THERAPY
TRANSPLANTS
VERTEBRATES
WHOLE-BODY IRRADIATION