| First Author | Novak E | Year | 1984 |
| Journal | Mouse News Lett | Volume | 71 |
| Pages | 46 | Mgi Jnum | J:14448 |
| Mgi Id | MGI:62616 | Citation | Novak E, et al. (1984) Correction of bleeding abnormalities in animal models of platelet storage pool deficiency by marrow transplantation. Mouse News Lett 71:46 |
| abstractText | Full text of MNL contribution: Correction of Bleeding Abnormalities in Animal Models of Platelet Storage Pool Deficiency by Marrow Transplantation. At least seven independent recessive gene mutations located on separate chromosomes in the mouse cause increased bleeding. Certain of these mutants are animal models for human storage pool diseases including Chediak-Higashi and Hermansky-Pudlak syndromes. These are maintained congenic with normal C57BL/6J except for the chromosomal site of the mutation. The phenotype of these mice includes prolonged bleeding, hypopigmentation, normal platelet counts and reduced numbers of platelet dense granules. The mutants differ among themselves in lysosomal secretion after thrombin stimulation. To test if the defect in the mutants is solely cellular, normal and congenic mutant mice were reciprocally transplanted with marrow after 950 rad whole body irradiation. The long bleeding times and abnormally low dense granule content in beige and pallid mutants were corrected when transplanted with normal marrow. Likewise, normal mice were converted to bleeders and had abnormally low dense contents when transplanted with mutant marrow. All transplanted mice had near normal blood platelet counts. We conclude that bleeding disorders in some murine storage pool disease models are cellular rather than humoral in origin and can be corrected by bone marrow transplantation. (E. Novak and R. Swank, with M. McGarry, Springville Laboratories). |
