| 背景信息 |
Deleted in primary ciliary dyskinesia homolog (mouse) (DPCD/Dpcd) encodes a small, conserved cytoplasmic protein implicated in the generation and maintenance of motile ciliated cells, with expression upregulated during ciliated cell differentiation in mouse airways and brain ependyma. Functional studies indicate that DPCD interacts with the R2TP co-chaperone complex and Akt kinase to regulate initiation of ciliogenesis, suggesting a role in organizing protein complexes needed for axoneme assembly and ciliary function. Mouse Dpcd shows broad developmental expression and annotated molecular functions that include cytoskeletal protein binding and carbohydrate derivative binding, consistent with participation in cilium-related structural and signaling pathways. In a classic mouse model, deletion of Dpcd underlies primary ciliary dyskinesia with defects in dynein inner arms, leading to impaired ciliary motility. Correspondingly, disruption of DPCD is linked to human ciliopathy phenotypes and has been associated with primary ciliary dyskinesia and related congenital disorders affecting mucociliary clearance. |