Amelioration of a Mouse Model of Osteogenesis Imperfecta with Hematopoietic Stem Cell Transplantation: Micro-Computed Tomography Studies

摘要

ObjectiveTo test the hypothesis that hematopoietic stem cells (HSCs) generate bone cells using bone marrow (BM) cell transplantation in a mouse model of osteogenesis imperfecta (OI). OI is a genetic disorder resulting from abnormal amount and/or structure of Type I collagen and is characterized by osteopenia, fragile bones and skeletal deformities. Homozygous OI murine mice (oim; B6C3Fe a/a-Col1a2^oim/J) offer excellent recipients for transplantation of normal HSCs, because fast turnover of osteoprogenitors has been shown.