Sickle cell disease promotes sex-dependent pathological bone loss through enhanced cathepsin proteolytic activity in mice.

ABSTRACT: Sickle cell disease (SCD) is the most common hereditary blood disorder in the United States. SCD is frequently associated with osteonecrosis, osteoporosis, osteopenia, and other bone-related complications such as vaso-occlusive pain, ischemic damage, osteomyelitis, and bone marrow hyperplasia known as sickle bone disease (SBD). Previous SBD models have failed to distinguish the age- and sex-specific characteristics of bone morphometry. In this study, we use the Townes mouse model of SCD to assess the pathophysiological complications of SBD in both SCD and sickle cell trait. Changes in bone microarchitecture and bone development were assessed by using high-resolution quantitative micro-computed tomography and the three-dimensional reconstruction of femurs from male and female mice. Our resu

SUBMITTER: Selma J 

PROVIDER: S-EPMC8905708 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature