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Updated: May 26, 2026

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08:46

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Ciliogenesis associated kinase 1 accumulates in its inactive form during polycystic kidney disease progression

Alice Serafin, Charlène Coquil, Aurore Dupuy

    Biorxiv : the Preprint Server for Biology
    |May 25, 2026
    PubMed

    Insights

    Ciliogenesis associated kinase 1 (CILK1) is overexpressed and accumulates in an inactive form in autosomal dominant polycystic kidney disease (ADPKD). This altered CILK1 regulation is linked to disease progression in ADPKD.

    Area of Science:

    • Nephrology
    • Molecular Biology
    • Genetics

    Background:

    • Ciliogenesis associated kinase 1 (CILK1) deficiency causes kidney developmental defects and cystogenesis.
    • Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease, but CILK1's role is unknown.
    • Understanding CILK1's function in ADPKD is crucial for potential therapeutic targets.

    Purpose of the Study:

    • To investigate the role and regulation of CILK1 in ADPKD.
    • To determine if CILK1 expression or activity is altered in ADPKD patient tissues and models.
    • To explore the relationship between CILK1 and disease progression in ADPKD.

    Main Methods:

    • Analysis of renal tissue from ADPKD patients and normal controls.
    • Utilizing mouse models of polycystic kidney disease.
    • Investigating CILK1 expression and phosphorylation status.
    • Examining the effect of Polycystic Kidney Disease 1 (PKD1) gene inactivation on CILK1.

    Main Results:

    • CILK1 is overexpressed in dedifferentiated cells of ADPKD renal tissue compared to controls.
    • Overexpressed CILK1 accumulates in a non-phosphorylated, inactive form.
    • Inactive CILK1 accumulation progresses with ADPKD.
    • Genetic inactivation of PKD1 triggers CILK1 accumulation.

    Conclusions:

    • CILK1 regulation is significantly altered in ADPKD.
    • Altered CILK1, particularly inactive forms, is a hallmark of ADPKD progression.
    • These findings suggest CILK1 as a potential biomarker or therapeutic target in ADPKD.

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