Osteogenesis Imperfecta: A Look into the Cerebellum of the Brtl Murine Model.

ABSTRACT: Osteogenesis imperfecta (OI), also known as brittle bone disease, is a rare congenital connective tissue disorder linked to collagen I defects, commonly known for its skeletal implications. However, collagen fibers play several key roles also in the central nervous system (CNS) starting from CNS development, regulating axonal growth, synaptogenesis, and terminal differentiation of Schwann cells. Collagen I plays an active role in defining brain architecture and in the developing peripheral. Furthermore, collagen is associated with astrogliosis and scar formation, processes finely regulated by molecules that are also involved in the regulation of the oxidative stress pathway. The aim of this work was to evaluate possible alterations in the cerebellum of the Brtl mouse, a well characterized

SUBMITTER: De Luca F 

PROVIDER: S-EPMC12559057 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature