Recombinant Human PKHD1L1 protein (GST tag N-Terminus)

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PKHD1L1 also known as Polycystic Kidney and Hepatic Disease 1-Like 1 is a large protein involved in cellular structures with a significant mechanical role. This protein has an approximate mass of 470 kDa. It is importantly expressed in tissues such as the kidney and liver indicating its role in these organs. PKHD1L1 is a part of the fibrocystin/polyductin complex that contributes to maintaining cellular integrity particularly in tissues that require structural support.

Biological function summary

The PKHD1L1 protein plays an essential role in maintaining cellular structural stability. It is an integral component of the fibrocystin/polyductin complex suggesting its involvement in structural cohesion within cells. Its presence in epithelial tissues such as the renal tubules and biliary ducts indicates it may also contribute to cellular architecture and fluid transport. This characteristic hints at roles in organ development and function particularly in the kidneys and liver systems.

Pathways

PKHD1L1 integrates into signaling pathways significant for maintaining cellular structure and function. It closely interacts within the primary cilia and polycystic kidney disease pathways where it influences cell proliferation and apoptosis processes. The relationship with fibrocystin a protein encoded by the PKHD1 gene highlights its role in cilia function and regular renal physiology associating it with important kidney pathways.

PKHD1L1 has a firm connection with polycystic kidney disease and liver fibrosis. These conditions involve abnormal cellular structure and function where PKHD1L1's mechanical role becomes evident. It connects to fibrocystin through its involvement in these disorders emphasizing their mutual impact on the development of cystic formations and fibrotic changes in the kidney and liver. By influencing these proteins and pathways PKHD1L1 contributes to the structural anomalies seen in these diseases.