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Mechanism of Ciliary Motion

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The ciliary structures were first seen in 1647 by Antonie Leeuwenhoek while observing the protozoans. In lower organisms, these appendages are responsible for cell movement, while in higher organisms, these appendages help in the movement of the extracellular fluids within the body cavities.
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...

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Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea

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Structural basis of the Inv compartment and ciliary abnormalities in Inv/nphp2 mutant mice.

Takuma Tsuji¹, Kazuhiko Matsuo¹, Takashi Nakahari²

¹Division of Anatomy and Developmental Biology, Department of Anatomy, Graduate School of Medicine, Kyoto Prefectural University of Medicine, Kyoto, 602-8566, Japan.

Cytoskeleton (Hoboken, N.J.)

|December 1, 2015

Summary

The Inv protein

Keywords:

Inv Inv compartment NPHP2 axoneme primary cilia

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Area of Science:

Cell Biology
Genetics
Biochemistry

Background:

Primary cilia are crucial hair-like organelles in mammalian cells, featuring a core axoneme structure.
The Inv gene is linked to nephronophthisis type 2 (NPHP2), a human kidney disease.
The Inv compartment is a specific region within cilia where the Inv protein localizes.

Purpose of the Study:

To investigate the structural basis of the Inv compartment in renal cilia.
To understand the role of the Inv protein in ciliary function and disease pathogenesis.
To compare Inv protein localization and function in different ciliated cell types.

Main Methods:

Comparative analysis of ciliary ultrastructure in renal and respiratory tissues.
Utilizing mouse models with mutations in the Inv gene.
Immunofluorescence microscopy to track Inv protein localization.

Main Results:

Renal cilia exhibit distinct proximal microtubule doublet and distal singlet regions.
The Inv compartment length correlates with the microtubule doublet region in renal cilia.
Inv-deficient respiratory cilia show structural defects and impaired ciliary beating, potentially explaining NPHP2 symptoms.
Inv protein is retained in the basal body of tracheal cells, not accumulating in the ciliary compartment.

Conclusions:

The microtubule doublet region may form the structural foundation of the Inv compartment in renal cilia.
Ciliary defects in Inv-deficient mice provide insights into NPHP2 pathogenesis.
Differential mechanisms govern Inv protein transport and retention in renal versus tracheal cilia.
Inv protein might have a distinct function within the basal body of tracheal cells.