Cilia Signaling in Development and Disease

1st Edition, Volume 155 - November 27, 2023
Editors: Carlo Iomini, Yang Sun
Language: English
Hardback ISBN:
9 7 8 - 0 - 1 2 - 8 2 0 1 5 9 - 6
eBook ISBN:
9 7 8 - 0 - 1 2 - 8 2 0 1 6 6 - 4

Cilia Signaling in Development and Disease, Volume 155 in the Current Topics in Developmental Biology series, highlights new advances in the field, with this new volume presen… Read more

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Cilia Signaling in Development and Disease, Volume 155 in the Current Topics in Developmental Biology series, highlights new advances in the field, with this new volume presenting interesting chapters on topics such as Cilia and mTOR signaling, Cilia and Kidney, Extraciliary functions of IFT proteins, Cilia and eye stem cells, Cilia and eye development, Cryo-EM/ET and cilia structure, and Control of protein and lipid composition of photoreceptor outer segments and the implications for eye diseases.

Cover image
Title page
Table of Contents
Series Page
Copyright
Contributors
Chapter One: Crosstalk between the mTOR pathway and primary cilia in human diseases
Abstract
1 Introduction
2 Structure of mTOR
3 Nutrient sensing mTOR signaling pathways
4 Primary cilia overview
5 Crosstalk between mTOR and Cilia
6 Role of mTOR and cilia signaling in kidney disease
7 Role of mTOR and cilia in brain development and diseases
8 mTOR and cilia in eye development and disease
9 Conclusion
References
Chapter Two: Functions of the primary cilium in the kidney and its connection with renal diseases
Abstract
1 Introduction
2 Ciliopathies
3 The primary cilium, the transition zone, and the formation of a specialized compartment
4 Ciliogenesis
5 Emergence of the primary cilium as a clinically important structure
6 Cilia and cell cycle regulation
7 Cilia regulation of signaling activity
8 Cilia and mechanosensation
9 Polycystic kidney diseases
10 Conclusion
References
Chapter Three: The complex relationship of Wnt-signaling pathways and cilia
Abstract
1 Introduction
2 Relationship of cilia and canonical Wnt/β-catenin signaling
3 Wnt–LRP6 signaling via the destruction complex/GSK3β promotes ciliary biogenesis
4 Wnt/Frizzled–PCP signaling regulates ciliary positioning and also centriole positioning in non-ciliated cells
5 Conclusions
Acknowledgments
References
Chapter Four: Patient stem cell-derived in vitro disease models for developing novel therapies of retinal ciliopathies
Abstract
1 Introduction
2 Structure of primary cilium
3 Photoreceptor outer segment as a specialized primary cilium
4 Retinal ciliopathies and their underlying gene mutations
5 Model systems to study ciliopathies
6 Methods for differentiation of retinal cells from human iPSCs
7 Characterization and staging of iPSC-derived retinal cells
8 Limitations of current methods
9 Mechanisms of human retinal ciliopathies using patient-derived cells
10 Approaches for treatment of retinal ciliopathies
11 Challenges in developing therapies using existing modalities
12 Conclusions and future outlook
Acknowledgments
References
Chapter Five: Control of protein and lipid composition of photoreceptor outer segments—Implications for retinal disease
Abstract
Abbreviations
1 Opening remarks
2 Part I: Overview of retinal function and disease—Placing the photoreceptor cilium into context
3 Part II. The many ways to disrupt outer segment structure and/or composition and their consequences for human vision
4 Summary and open questions
References

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Carlo Iomini

Yang Sun