Mouse Dynein Intermediate Chain 2, Axonemal (DNAI2) ELISA Kit

Background

Dynein axonemal intermediate chain 2 (DNAI2) is a crucial component of the axonemal dynein complex, specifically associated with the outer dynein arms of motile cilia and flagella. Like DNAI1, mutations in DNAI2 are a common cause of primary ciliary dyskinesia (PCD), manifesting as defective ciliary motion, leading to symptoms such as chronic sinusitis, otitis media, bronchiectasis, and male infertility. DNAI2 is integral to the attachment and stabilization of the outer dynein arm to the axonemal microtubules. Functional assays indicate that loss-of-function mutations in DNAI2 often result in a near-complete absence of outer dynein arms, reducing the amplitude and frequency of ciliary beating. DNAI2 interacts with other intermediate and heavy chain proteins within the dynein complex, such as DNAH5 and DNAI1, to ensure coordinated assembly during ciliogenesis. In vitro studies show that defects in DNAI2 impair mucociliary clearance mechanisms, significantly affecting respiratory health. DNAI2 expression is tissue-specific, primarily found in motile ciliated epithelia and sperm flagella. It has a role in dynein arm preassembly, relying on cytoplasmic assembly factors like DNAAF proteins, which transport dynein subunits to the growing axoneme. Structural insights into DNAI2 reveal conserved domains essential for microtubule binding and ATPase activity regulation, both of which are required for dynein-driven motility. Model organisms deficient in DNAI2 exhibit phenotypes closely resembling human PCD, including situs inversus and chronic airway inflammation. Current studies suggest that DNAI2 mutations may also affect dynein complex stability, contributing to progressive ciliary dysfunction. DNAI2 plays a significant role in ensuring the dynamic adaptability of ciliary motion, particularly in response to environmental stimuli.