Cited by 239Lysosomal Storage Disorders ResearchAmyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatments

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The lysosomal function of progranulin, a guardian against neurodegeneration

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Paushter, Daniel H., Du, Huan, Feng, Tuancheng, Hu, Fenghua·2018·Acta Neuropathologica

DOI 10.1007/s00401-018-1861-8PMC PMC6117207

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Abstract

Progranulin (PGRN), encoded by the GRN gene in humans, is a secreted growth factor implicated in a multitude of processes ranging from regulation of inflammation to wound healing and tumorigenesis. The clinical importance of PGRN became especially evident in 2006, when heterozygous mutations in the GRN gene, resulting in haploinsufficiency, were found to be one of the main causes of frontotemporal lobar degeneration (FTLD). FTLD is a clinically heterogenous disease that results in the progressive atrophy of the frontal and temporal lobes of the brain. Despite significant research, the exact function of PGRN and its mechanistic relationship to FTLD remain unclear. However, growing evidence suggests a role for PGRN in the lysosome-most striking being that homozygous GRN mutation leads to neuronal ceroid lipofuscinosis, a lysosomal storage disease. Since this discovery, several links between PGRN and the lysosome have been established, including the existence of two independent lysosomal trafficking pathways, intralysosomal processing of PGRN into discrete functional peptides, and direct and indirect regulation of lysosomal hydrolases. Here, we summarize the cellular functions of PGRN, its roles in the nervous system, and its link to multiple neurodegenerative diseases, with a particular focus dedicated to recent lysosome-related mechanistic developments.

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Authors: Paushter, D. H., Du, H., Feng, T., & Hu, F.
Affiliations: Cornell University (US)
Corresponding author: Hu, Fenghua
ORCID: Du, Huan
Feng, Tuancheng
Hu, Fenghua
Venue: Acta Neuropathologica
Publisher: Springer Science and Business Media LLC
Published: 2018-05-09
Volume: 136
Issue: 1
Pages: 1-17
ISSN: 0001-6322 (ISSN-L)
1432-0533
DOI: 10.1007/s00401-018-1861-8
PMC: PMC6117207
OpenAlex: W2802961407
Cited by: 230
References: 161
Citations by year: 2026: 21 · 2025: 31 · 2024: 33 · 2023: 32 · 2022: 26 · 2021: 45 · 2020: 19 · 2019: 18 · 2018: 3
FWCI: 2.79
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
www.springer.com/tdm (vor)
Locations: Acta Neuropathologica (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute of Neurological Disorders and Stroke (R01NS088448, R01NS095954)
Topics: Health Sciences › Medicine › Genetics (clinical) › Lysosomal Storage Disorders Research
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: progranulin, lysosomes, lysosomal trafficking, frontotemporal lobar degeneration, haploinsufficiency, neuronal ceroid lipofuscinosis, lysosomal storage disease, lysosomal enzymes, neurodegeneration, neurodegenerative diseases
MeSH terms: Progranulins, Animals, Humans, Lysosomes, Models, Biological, Mutation, Neurodegenerative Diseases, Protein Transport

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The lysosomal function of progranulin, a guardian against neurodegeneration

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