Leucine-Rich Repeat-Containing Protein 48 (LRRC48) Antibody

Background

DRC3 is a component of the dynein regulatory complex (DRC), a critical structure within motile cilia and flagella that regulates the activity of axonemal dynein, ensuring proper ciliary beating and motility. DRC3 contributes to the stability and organization of the nexin-dynein regulatory complex, which links outer doublet microtubules and regulates dynein motor activity for coordinated ciliary motion. It is highly expressed in ciliated tissues, such as respiratory epithelium, reproductive organs, and brain ependyma, where it ensures efficient mucus clearance, fluid movement, and sperm motility. Mutations in DRC3 are associated with primary ciliary dyskinesia (PCD), a disorder characterized by impaired ciliary beating, leading to chronic respiratory infections, reduced fertility, and hydrocephalus due to defective fluid flow in the brain. Functional studies show that DRC3 is essential for maintaining ciliary structure and dynein regulation, as its loss causes abnormal ciliary waveform, reduced beat frequency, and impaired motility, highlighting its crucial role in the regulation of ciliary function and cellular homeostasis.