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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.
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