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KLC3 regulates axonemal glutamylation via RAB11/FIP5 in polycystic kidney disease

Gyuyeong Rah1,2, Je Yeong Ko1,3, Yejin Ahn1,3

  • 1Department of Biological Sciences, Sookmyung Women's University, 100, Cheongpa-ro 47-gil, Yongsan-gu, Seoul, Republic of Korea.

Cell Communication and Signaling : CCS
|November 13, 2025
PubMed

Insights

Kinesin Light Chain 3 (KLC3) regulates tubulin modifications in primary cilia, impacting cyst progression in polycystic kidney disease (PKD). This study reveals KLC3

Area of Science:

  • Cell Biology
  • Genetics
  • Nephrology

Background:

  • Polycystic kidney disease (PKD) is characterized by cyst formation due to defects in primary cilia of cyst-lining cells.
  • KLC3 was previously identified as a novel ciliary gene implicated in PKD pathogenesis.
  • RAB11FIP5 is known to regulate tubulin post-translational modifications (PTM) within primary cilia.

Purpose of the Study:

  • To elucidate the relationship between KLC3 and RAB11FIP5 in mediating tubulin PTM.
  • To investigate the role of this interaction in the progression of PKD.

Main Methods:

  • Co-localization and interaction studies of KLC3 and RAB11FIP5 at primary cilia basal bodies.
  • Analysis of KLC3's effect on axonemal glutamylation and RAB11FIP5 expression.
  • Assessment of KLC3, ciliary glutamylation, IFT88, and cell proliferation in ADPKD patient-derived cells.

Main Results:

  • KLC3 co-localizes and interacts with RAB11FIP5 around primary cilia basal bodies.
  • KLC3 regulates axonemal glutamylation, influencing RAB11FIP5 expression and potentially anterograde ciliary trafficking.
  • Increased RAB11FIP5 and axonemal glutamylation observed in PKD cyst-lining cells, mirroring KLC3 findings.
  • Decreased KLC3 in ADPKD cells led to reduced ciliary glutamylation, IFT88 levels, and cell proliferation.

Conclusions:

  • KLC3 regulates ciliary glutamylation through RAB11FIP5.
  • This KLC3-RAB11FIP5-mediated pathway may be a significant contributor to cyst progression in PKD.
  • Targeting this pathway could offer therapeutic potential for PKD treatment.
Keywords:
Kinesin light chain-3Polycystic kidney diseasePrimary ciliaRAB11FIP5Tubulin post-translational modification

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