Leucine-Rich Repeat-Containing Protein 6 (LRRC6) Antibody

Background

DNAAF11, also known as LRRC6, is a dynein axonemal assembly factor essential for the preassembly and transport of axonemal dynein arms into motile cilia. It interacts with chaperone proteins and cytoplasmic complexes to facilitate the proper folding, assembly, and localization of dynein motor components, ensuring the beating of motile cilia and flagella. DNAAF11 is expressed in ciliated tissues, including the respiratory tract, reproductive organs, and brain ependyma, where it regulates processes like mucus clearance, sperm motility, and cerebrospinal fluid flow. Mutations in DNAAF11 result in primary ciliary dyskinesia (PCD), a disorder characterized by immotile or dyskinetic cilia, leading to chronic respiratory infections, infertility, and hydrocephalus. Functional studies reveal that DNAAF11 deficiency disrupts dynein arm assembly, causing structural defects in cilia and impaired motility, underscoring its essential role in axonemal dynein regulation, ciliary function, and human health.