Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration

Abstract Background Progranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP-Pgrn). Progranulin (PGRN) is an intracellular and secreted pro-protein that is proteolytically cleaved into individual granulin peptides, which are inc...

書目詳細資料
發表在:	Molecular Neurodegeneration
Main Authors:	Swetha Mohan, Paul J. Sampognaro, Andrea R. Argouarch, Jason C. Maynard, Mackenzie Welch, Anand Patwardhan, Emma C. Courtney, Jiasheng Zhang, Amanda Mason, Kathy H. Li, Eric J. Huang, William W. Seeley, Bruce L. Miller, Alma Burlingame, Mathew P. Jacobson, Aimee W. Kao
格式:	Article
語言:	英语
出版:	BMC 2021-08-01
主題:	Progranulin Granulin Frontotemporal lobar degeneration Lysosome Protease pH
在線閱讀:	https://doi.org/10.1186/s13024-021-00472-1

相似書籍

Deletion of the progranulin gene in patients with frontotemporal lobar degeneration or Parkinson disease
由: Anne Rovelet-Lecrux, et al.
出版: (2008-07-01)

Reduction of sphingomyelinase activity associated with progranulin deficiency and frontotemporal dementia
由: Nicholas R. Boyle, et al.
出版: (2025-09-01)

Clinical and Imaging Features of Sporadic and Genetic Frontotemporal Lobar Degeneration TDP‐43 A and B
由: Sean Coulborn, et al.
出版: (2025-05-01)

Microglial progranulin differently regulates hypothalamic lysosomal function in lean and obese conditions via cleavage-dependent mechanisms
由: Chae Beom Park, et al.
出版: (2025-03-01)

Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice
由: Julia K. Götzl, et al.
出版: (2018-09-01)

Response to: Clinical Imaging Features of Sporadic and Genetic Frontotemporal Lobar Degeneration TDP‐43 A and B
由: Sergi Borrego‐Écija, et al.
出版: (2025-10-01)

Frontotemporal Lobar Degeneration and microRNAs
由: Paola ePiscopo, et al.
出版: (2016-02-01)

The lysosomal protein cathepsin L is a progranulin protease
由: Chris W. Lee, et al.
出版: (2017-07-01)

Genetics of frontotemporal lobar degeneration
由: Aswathy P, et al.
出版: (2010-10-01)

Anti-sortilin1 Antibody Up-Regulates Progranulin via Sortilin1 Down-Regulation
由: Shuuichi Miyakawa, et al.
出版: (2020-12-01)

Progranulin regulates neuronal outgrowth independent of Sortilin
由: Gass Jennifer, et al.
出版: (2012-07-01)

New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
由: Hee Kyung Park, et al.
出版: (2013-04-01)

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia
由: Wonjae Sung, et al.
出版: (2024-02-01)

Pharmacological treatment of frontotemporal lobar degeneration: systematic review Tratamento farmacológico da degeneração lobar frontotemporal: revisão sistemática
由: Maria da Glória Portugal, et al.
出版: (2011-03-01)

GRN−/− iPSC-derived cortical neurons recapitulate the pathological findings of both frontotemporal lobar degeneration and neuronal ceroidolipofuscinosis
由: Patrizia Bossolasco, et al.
出版: (2022-12-01)

Subcortical and Deep Cortical Atrophy in Frontotemporal Dementia due to Granulin Mutations
由: Enrico Premi, et al.
出版: (2014-04-01)

Parkin Levels Decrease in Fibroblasts With Progranulin (PGRN) Pathogenic Variants and in a Cellular Model of PGRN Deficiency
由: Katarzyna Gaweda-Walerych, et al.
出版: (2021-05-01)

A case of familial frontotemporal dementia caused by a progranulin gene mutation
由: Lauryn Currens, et al.
出版: (2023-01-01)

The role of endolysosomal progranulin and TMEM106B in neurodegenerative diseases
由: Hideyuki Takahashi, et al.
出版: (2025-07-01)

Co-Occurrence of Language and Behavioural Change in Frontotemporal Lobar Degeneration
由: Jennifer M. Harris, et al.
出版: (2016-06-01)

Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice
由: Terri L. Petkau, et al.
出版: (2017-10-01)

Molecular Pathways Bridging Frontotemporal Lobar Degeneration and Psychiatric Disorders
由: Roberta eZanardini, et al.
出版: (2016-02-01)

A systematic review of progranulin concentrations in biofluids in over 7,000 people—assessing the pathogenicity of GRN mutations and other influencing factors
由: Imogen J. Swift, et al.
出版: (2024-03-01)

HnRNP Pathologies in Frontotemporal Lobar Degeneration
由: Xinwa Jiang, et al.
出版: (2023-06-01)

Clinical Characteristics of C9ORF72-Linked Frontotemporal Lobar Degeneration
由: Anna-Lotta Kaivorinne, et al.
出版: (2013-08-01)

Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome
由: Aya Murakami, et al.
出版: (2025-07-01)

Granulins Regulate Aging Kinetics in the Adult Zebrafish Telencephalon
由: Alessandro Zambusi, et al.
出版: (2020-02-01)

The underappreciated contribution of Oskar Fischer to the knowledge of Frontotemporal Lobar Degeneration
由: Eliasz Engelhardt, et al.
出版: (2025-02-01)

Progranulin Deficiency Induces Mitochondrial Dysfunction in Frontotemporal Lobar Degeneration with TDP-43 Inclusions
由: Guiomar Rodríguez-Periñán, et al.
出版: (2023-02-01)

Assessing network degeneration and phenotypic heterogeneity in genetic frontotemporal lobar degeneration by decoding FDG-PET
由: Nick Corriveau-Lecavalier, et al.
出版: (2024-01-01)

Autoimmunity and Frontotemporal Lobar Degeneration: From Laboratory Study to Clinical Practice
由: Sun Y, et al.
出版: (2023-03-01)

Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach
由: Genaro Gabriel Ortiz, et al.
出版: (2023-10-01)

Neuropsychological features of progranulin-associated frontotemporal dementia: a nested case-control study
由: Marisa Lima, et al.
出版: (2021-01-01)

Performance of patients with frontotemporal lobar degeneration on artistic tasks: A pilot study
由: Maria Cristina Anauate, et al.

Blood Biomarkers in Frontotemporal Dementia: Review and Meta-Analysis
由: Sofia Ntymenou, et al.
出版: (2021-02-01)

Epigenetic Age Acceleration in Frontotemporal Lobar Degeneration: A Comprehensive Analysis in the Blood and Brain
由: Megha Murthy, et al.
出版: (2023-07-01)

Human progranulin-expressing mice as a novel tool for the development of progranulin-modulating therapeutics
由: Terri L. Petkau, et al.
出版: (2021-06-01)

Folding of Truncated Granulin Peptides
由: Rozita Takjoo, et al.
出版: (2020-08-01)

CSF neurogranin levels as a biomarker in Alzheimer’s disease and frontotemporal lobar degeneration: a cross-sectional analysis
由: Vanesa Jurasova, et al.
出版: (2024-09-01)

Excessive expression of progranulin leads to neurotoxicity rather than neuroprotection
由: Shinya Kusakari, et al.
出版: (2025-06-01)