重组人FOPNL蛋白

补充信息

This supplementary information is collated from multiple sources and compiled automatically.

FOPNL also known as FGFR1OP N-terminal-like protein is a coiled-coil protein with a mass of about 37 kDa. This protein localizes primarily in the cytoplasm and has roles within cellular structures like centrioles. Expression of FOPNL is seen across multiple tissues suggesting its involvement in fundamental cellular processes. By interacting with key structural components within the cell it contributes to significant cellular functions.

Biological function summary

FOPNL interacts with other proteins to stabilize centrosome dynamics and is an integral part of the BBSome complex important in ciliary function. The BBSome complex identified in ciliated cells plays a significant role in the correct targeting and trafficking of proteins to the primary cilium. By ensuring proper ciliary function FOPNL influences cellular signaling and sensory roles vital for maintaining various cellular processes.

Pathways

FOPNL participates in cellular mechanisms like the BBSome transport pathway and centrosome-related signaling. It interacts closely with proteins such as BBS1 and BBS4 which are part of the BBSome complex regulating the transport of proteins through ciliary gates. The positioning of FOPNL within these pathways emphasizes its regulatory role in maintaining effective intracellular communication and centrosomal stability.

Defects in FOPNL function link to Bardet-Biedl syndrome a condition marked by multiorgan involvement. Mutations affecting FOPNL disrupt the integrity of the BBSome leading to impaired ciliary function and subsequently the clinical manifestations of the syndrome. Additionally interactions involving proteins such as CEP290 further connect FOPNL to this disorder highlighting the collaborative role these proteins play in stabilizing structural cellular components to prevent disease development.