Characterization of Bone and Brain Abnormalities Associated with Sickle Cell Disease

Sickle Cell Disease (SCD) is one of the most severe, monogenic, autosomal recessive disorders in the world. SCD causes devastating pain, inflammation, organ damage, vaso-occlusion and bone damage. The Berkeley mouse model is useful for the evalutation of the disease because the animals present symptoms similar to the human condition. The data presented in this study demostrate that SCD causes bone abnormalities in the Berkeley mouse model similar to those present in humans. Specifically, SCD causes significant decreases in bone mineral density and bone mineral content of the BERK mice in the whole body, cervical and lumbar vertebrae and femur. Also, peripheral inflammation associated with SCD increases the permeability of the blood brain barrier and enables infiltration of monocytes into the brain. Although the BERK model has been characterized extensively, SCD effects on bone structure and the ability of immune cells to permeate the brain have not been described