Evidence map›Paper›PMID 40528203›Full record

ArticleMolecular neurodegeneration2025

sPLA2-IIA modifies progranulin deficiency phenotypes in mouse models.

Cha Yang, Huan Du, Gwang Bin Lee, Masaaki Uematsu, Weiguo He, Etienne Doré, Weizhi Yu, Ethan J Sanford, Marcus B Smolka, Eric Boilard and 3 more

Abstract read
In one paragraph

Article in Molecular neurodegeneration, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
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1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

13 authors.

Cha Yang *Department of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA.
Huan Du *Department of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA.
Gwang Bin LeeDepartment of Chemistry and Biochemistry, University of Maryland, College Park, MD, 20742, USA.
Masaaki UematsuDepartment of Chemistry and Chemical Biology, Ithaca, NY, 14853, USA.
Weiguo HeDepartment of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA.
Etienne DoréCentre de Recherche du Centre Hospitalier Universitaire de Québec, Centre de Recherche ARThrite - Arthrite, Recherche, Traitements, Université Laval, Québec, QC, Canada.
Weizhi YuDepartment of Chemistry and Chemical Biology, Ithaca, NY, 14853, USA.
Ethan J SanfordDepartment of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA.
Marcus B SmolkaDepartment of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA.
Eric BoilardCentre de Recherche du Centre Hospitalier Universitaire de Québec, Centre de Recherche ARThrite - Arthrite, Recherche, Traitements, Université Laval, Québec, QC, Canada.
Jeremy M BaskinDepartment of Chemistry and Chemical Biology, Ithaca, NY, 14853, USA.
Ling HaoDepartment of Chemistry and Biochemistry, University of Maryland, College Park, MD, 20742, USA.
Fenghua HuDepartment of Molecular Biology and Genetics, 345 Weill Hall, Ithaca, NY, 14853, USA. fh87@cornell.edu.

Funding

Lysosomal Function of Progranulin and NeurodegenerationR01NS095954 · NINDS · CORNELL UNIVERSITY · PI Fenghua Hu · 2017 to 2026
$4.9M
Function of TMEM106B in NeurodegenerationR01NS088448 · NINDS · CORNELL UNIVERSITY · PI HU, FENGHUA · 2014 to 2025
$4.2M
Development of Mass Spectrometry Strategies to Decipher Dynamic Lysosomal Dysfunctions in Frontotemporal DementiaR01NS121608 · NINDS · UNIV OF MARYLAND, COLLEGE PARK · PI Ling Hao · 2021 to 2026
$2.0M
Spatiotemporal control of ubiquitination by phosphoinositide-binding proteinsR01GM143367 · NIGMS · CORNELL UNIVERSITY · PI BASKIN, JEREMY · 2022 to 2025
$1.3M
Investigating the role of progranulin in TDP-43 proteinopathyR21AG078741 · NIA · CORNELL UNIVERSITY · PI HU, FENGHUA · 2022 to 2022
$449k
NIA NIH HHS R21 AG078741NIGMS NIH HHS R01 GM143367NINDS NIH HHS R01 NS088448NINDS NIH HHS R01 NS095954NINDS NIH HHS R01NS095954NINDS NIH HHS R01 NS121608
6 · The paper itself

Abstract

backgroundHaploinsufficiency of the progranulin (PGRN) protein is a leading cause of frontotemporal lobar degeneration (FTLD). Mouse models have been developed to study PGRN functions. However, PGRN deficiency in the commonly used C57BL/6 mouse strain background leads to very mild phenotypes, and pathways regulating PGRN deficiency phenotypes remain to be elucidated.

methodsWe generated PGRN-deficient mice in the FVB/N background and compared PGRN deficiency phenotypes between C57BL/6 and FVB/N backgrounds via immunostaining, western blot, RNA-seq, and proteomics approaches. We demonstrated a novel pathway in modifying PGRN deficiency phenotypes using inhibitor treatment and AAV-mediated overexpression in mouse models.

resultsWe report that PGRN loss in the FVB/N mouse strain results in earlier onset and stronger FTLD-related and lysosome-related phenotypes. We found that PGRN interacts with sPLA2-IIA, a member of the secreted phospholipase A2 (sPLA2) family member and a key regulator of inflammation, that is expressed in FVB/N but not C57BL/6 background. sPLA2-IIA inhibition rescues PGRN deficiency phenotypes, while sPLA2-IIA overexpression drives enhanced gliosis and lipofuscin accumulation in PGRN-deficient mice. Additionally, RNA-seq and proteomics analysis revealed that mitochondrial pathways are upregulated in the PGRN-deficient C57BL/6 mice but not in the FVB/N mice.

conclusionsOur studies establish a better mouse model for FTLD-GRN and uncover novel pathways modifying PGRN deficiency phenotypes.

Indexed as

Frontotemporal Lobar DegenerationProgranulinsAnimalsDisease Models, AnimalMiceMice, Inbred C57BLPhenotypeGrn protein, mouseProgranulinsInflammationLysosomeMitochondriaMouse strain backgroundProgranulinsPLA2-IIA

Identifiers

PMID40528203
PMCPMC12175356

What Socratic holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.