التفاصيل البيبلوغرافية
| العنوان: |
Progranulinopathy: A diverse realm of disorders linked to progranulin imbalances. |
| المؤلفون: |
Huang, Guiwu1,2,3 (AUTHOR), Jian, Jinlong1,2 (AUTHOR), Liu, Chuan-Ju1,2,4 (AUTHOR) chuan-ju.liu@yale.edu |
| المصدر: |
Cytokine & Growth Factor Reviews. Apr2024, Vol. 76, p142-159. 18p. |
| مصطلحات موضوعية: |
*PROGRANULIN, *LYSOSOMAL storage diseases, *PERIPHERAL nervous system, *PROTEIN microarrays, *MUSCULOSKELETAL system diseases, *DEGENERATION (Pathology) |
| مستخلص: |
Progranulin (PGRN), encoded by the GRN gene in humans, was originally isolated as a secreted growth factor that implicates in a multitude of processes ranging from regulation of tumorigenesis, inflammation to neural proliferation. Compelling evidence indicating that GRN mutation can lead to various common neuronal degenerative diseases and rare lysosomal storage diseases. These findings have unveiled a critical role for PGRN as a lysosomal protein in maintaining lysosomal function. The phenotypic spectrum of PGRN imbalance has expanded to encompass a broad spectrum of diseases, including autoimmune diseases, metabolic, musculoskeletal and cardiovascular diseases. These diseases collectively referred to as Progranulinopathy- a term encompasses the wide spectrum of disorders influenced by PGRN imbalance. Unlike its known extracellular function as a growth factor-like molecule associated with multiple membrane receptors, PGRN also serves as an intracellular co-chaperone engaged in the folding and traffic of its associated proteins, particularly the lysosomal hydrolases. This chaperone activity is required for PGRN to exert its diverse functions across a broad range of diseases, encompassing both the central nervous system and peripheral systems. In this comprehensive review, we present an update of the emerging role of PGRN in Progranulinopathy, with special focus on elucidating the intricate interplay between PGRN and a diverse array of proteins at various levels, ranging from extracellular fluids and intracellular components, as well as various pathophysiological processes involved. This review seeks to offer a comprehensive grasp of PGRN's diverse functions, aiming to unveil intricate mechanisms behind Progranulinopathy and open doors for future research endeavors. [Display omitted] • Progranulin is involved in processes ranging from tumorigenesis and inflammation to neural proliferation. • Mutations in the GRN gene can result in various neuronal degenerative diseases and rare lysosomal storage disorders. • The range of conditions and diseases stemming from PGRN imbalance collectively termed as Progranulinopathy. • Beyond recognized role as an extracellular growth factor-like molecule, PGRN also operates as an intracellular co-chaperone. • This review delves into the complex interplay between PGRN and an assorted array of proteins across different tiers. [ABSTRACT FROM AUTHOR] |
| قاعدة البيانات: |
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