LB965 Sonic hedgehog signaling promotes aberrant hair follicle stem cell differentiation and subcutaneous ossification formation in a mouse model of albright hereditary osteodystrophy

Abstract

Albright hereditary osteodystrophy (AHO) is a disorder caused by GNAS heterozygous inactivation, and results in the spontaneous development of subcutaneous ossifications (SCOs) in the dermis and subcutaneous tissue. We have generated an AHO mouse model through global Gnas heterozygous inactivation (Gnas+/-) that phenocopies the human disorder and develops SCOs that consistently surround hair follicles (HF). Therefore, this study examined the cellular and molecular etiologies contributing to SCO formation within the HF microenvironment of Gnas+/- mice.