ACVR1抗体

The ACVR1 antibody targets the Activin A receptor type 1 (ACVR1), a transmembrane serine/threonine kinase receptor involved in the bone morphogenetic protein (BMP) signaling pathway. ACVR1 plays a critical role in regulating cellular processes such as proliferation, differentiation, and apoptosis, particularly in skeletal development and tissue homeostasis. Dysregulation of ACVR1 is linked to fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder characterized by progressive heterotopic ossification. In FOP, a recurrent gain-of-function mutation (R206H) in ACVR1 causes constitutive activation of BMP signaling, even in the absence of ligands, leading to abnormal bone formation. ACVR1 antibodies are essential tools for studying this receptor's expression, activation mechanisms, and pathological roles. They enable detection of ACVR1 in tissues or cell lines, assess phosphorylation status, and evaluate therapeutic interventions. Additionally, neutralizing antibodies are being explored to inhibit aberrant BMP signaling in FOP, offering potential therapeutic strategies. Beyond FOP, ACVR1 antibodies contribute to research in cancer, as ACVR1 mutations or altered expression are implicated in diffuse intrinsic pontine glioma (DIPG) and other malignancies. These antibodies thus serve both diagnostic and research purposes, bridging mechanistic insights into ACVR1-related diseases and therapeutic development.