Article

Targeting complement C3a receptor resolves mitochondrial hyperfusion and subretinal microglial activation in progranulin-deficient frontotemporal dementia

2024-06-01

Abstract excerpt

<h4>SUMMARY</h4> Mutations in progranulin ( GRN ) cause frontotemporal dementia ( GRN -FTD) due to deficiency of the pleiotropic protein progranulin. GRN -FTD exhibits diverse pathologies including lysosome dysfunction, lipofuscinosis, microgliosis, and neuroinflammation. Yet, how progranulin loss causes disease remains unresolved. Here, we report that non-invasive retinal imaging of GRN -FTD patients reveale...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: e4b95ee4-a2fe-5c10-bd77-b99ff87a1f96
DOI: 10.1101/2024.05.29.595206

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Targeting complement C3a receptor resolves mitochondrial hyperfusion and subretinal microglial activation in progranulin-deficient frontotemporal dementiaDOI 10.1101/2024.05.29.595206Read

Progranulin deficiency in the brain: the interplay between neuronal and non-neuronal cellsSemantic proximity Read

Transplantation of Human IPSC-derived Microglia Ameliorates Neuropathology and Circuit Dysfunction in Progranulin-Deficient MiceSemantic proximity Read

Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.Semantic proximity Read

Transplantation of Human IPSC-derived Microglia Ameliorates Neuropathology and Circuit Dysfunction in Progranulin-Deficient MiceSemantic proximity Read

Opposing role of phagocytic receptors MERTK and AXL in Progranulin deficient FTD.Semantic proximity Read

Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microgliaSemantic proximity Read

Progranulin Gene Therapy Improves Lysosomal Dysfunction and Microglial Pathology Associated with Frontotemporal Dementia and Neuronal Ceroid LipofuscinosisSemantic proximity Read

Multi-omic analysis of photoreceptor alterations during early-onset retinal degeneration in <i> Mfrp <sup>-/-</sup> </i> miceSemantic proximity Read

Progranulin deficiency induces lipid droplet accumulation in microglia via a STAT3-GPAT3 axisSemantic proximity Read

New insights and therapeutic opportunities for progranulin-deficient frontotemporal dementia.Semantic proximity Read

Biochemical, biomarker, and behavioral characterization of the <i> Grn <sup>R493X</sup> </i> mouse model of frontotemporal dementiaSemantic proximity Read

Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis.Semantic proximity Read