Progranulin and Central Nervous System Disorders

  • Hideaki Hara
  • Masato Hosokawa
  • Shinsuke Nakamura
  • Takayoshi Shimohata
  • Masugi Nishihara

Table of contents

  1. Front Matter
    Pages i-vii
  2. Akio Kimura, Masao Takemura, Takayoshi Shimohata
    Pages 19-34
  3. Masato Hosokawa, Tetsuaki Arai
    Pages 35-69
  4. Masato Hosokawa
    Pages 117-126
  5. Masato Kanazawa, Kunio Kawamura, Tetsuya Takahashi, Takayoshi Shimohata
    Pages 157-167
  6. Yoshiki Kuse, Shinsuke Nakamura, Hideaki Hara
    Pages 169-183

About this book


This book presents the latest knowledge on the roles of progranulin (PGRN) in normal physiology and pathology and explores the emerging significance of PGRN as a therapeutic target and biomarker in various CNS disorders, including frontotemporal lobe degeneration and other neurodegenerative diseases. Following initial recognition of the importance of PGRN in sexual differentiation of the developing brain and adult neurogenesis, it was subsequently discovered that PGRN acts as a chaperone of lysosomal enzymes and plays a crucial role in maintaining cellular protein homeostasis. It has also been found that sex steroids modulate the expression of PGRN and its trophic effects in the developing CNS and that PGRN directly or indirectly influences neural stem and progenitor cells. Against this background, deeper understanding of the molecular and functional properties of PGRN would provide fresh impetus for the development of mechanism-based therapeutic approaches for multiple disorders. Medications targeting the recovery of lysosomal function appear to hold particular promise in patients with neurodegenerative diseases resulting from PGRN insufficiency. In examining multiple aspects of this fascinating field, the book will be of high value for researchers and graduate students.


Progranulin Lysosome Neurodegeneration Neurogenesis Frontotemporal lober degeneration Brain Estrogen Extracellular matrix Retina

Editors and affiliations

  • Hideaki Hara
    • 1
  • Masato Hosokawa
    • 2
  • Shinsuke Nakamura
    • 3
  • Takayoshi Shimohata
    • 4
  • Masugi Nishihara
    • 5
  1. 1.Molecular Pharmacology, Department of Biofunctional EvaluationGifu Pharmaceutical UniversityGifuJapan
  2. 2.Dementia Research Project, Department of Dementia and Higher Brain FunctionTokyo Metropolitan Institute of Medical ScienceTokyoJapan
  3. 3.Molecular Pharmacology, Department of Biofunctional EvaluationGifu Pharmaceutical UniversityGifuJapan
  4. 4.Department of NeurologyGifu University Graduate School of MedicineGifuJapan
  5. 5.Department of Veterinary Physiology, Graduate School of Agricultural and Life SciencesThe University of TokyoTokyoJapan

Bibliographic information