Anti-OSTM1 antibody

Supplementary info

Activity summary

OSTM1 also known as Osteopetrosis Associated Transmembrane Protein 1 is a protein with a molecular mass of approximately 47 kDa. It primarily resides in lysosomal and endosomal membranes. OSTM1 is highly expressed in osteoclasts cells responsible for bone resorption. It is important for osteoclast differentiation and function ensuring the breakdown of the bone matrix. OSTM1 forms a complex with other membrane proteins assisting in ion homeostasis within the osteoclast environment.

Biological function summary

The OSTM1 protein contributes to maintaining cellular ionic balance and is a part of the chloride channel complex. This complex is important for proper osteoclast activity as it facilitates the transport of ions necessary for creating an acidic environment. The protein participates in chloride-ion channel transport affecting the acidification of resorptive vacuoles. This action supports bone resorption enabling bone remodeling and growth.

Pathways

OSTM1 plays an important role in the RANK-RANKL signaling pathway which regulates osteoclast differentiation and activation. OSTM1 interacts with proteins like CLCN7 another chloride channel protein within this pathway. Through this interaction it assists in the formation and maintenance of acidic compartments in osteoclasts essential for bone mineral dissolution. Therefore OSTM1 is integral to the processes of bone turnover and mineral homeostasis.

Associated diseases and disorders

Mutations or malfunctions in OSTM1 are directly linked to the development of autosomal recessive osteopetrosis a condition characterized by increased bone density and associated complications. OSTM1 dysfunction leads to impaired osteoclast activity resulting in defective bone resorption. Additionally a connection exists between OSTM1 and CLCN7 where defects in either protein can manifest similar clinical conditions highlighting their collaborative role in maintaining skeletal health.