Article

Seizures in PPT1 Knock-in Mice are Associated with Inflammatory Activation of Microglia

2022-03-25

Abstract excerpt

<title>Abstract</title> <p>Infantile neuronal ceroid lipofuscinosis (INCL), the most severe form of neuronal ceroid lipofuscinoses, is caused by mutations in the lysosomal enzyme palmitoyl protein thioesterase 1 (PPT1). Typical symptoms of this disease include progressive psychomotor developmental retardation, visual failure, seizures, and premature death. Here, we investigated seizure activity and relevant patho...

Topics

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

Identifiers and source

Literature Corpus work: 3047c058-fe46-5304-8d83-162fbdff1eca
DOI: 10.21203/rs.3.rs-1480352/v1

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Seizures in PPT1 Knock-in Mice are Associated with Inflammatory Activation of MicrogliaDOI 10.21203/rs.3.rs-1480352/v1Read

Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?Semantic proximity Read

PPT1 Deficiency-Induced GABAAR Hyperpalmitoylation Impairs Synaptic Transmission and Memory FormationSemantic proximity Read

The Role of Attenuated Astrocyte Activation in Infantile Neuronal Ceroid LipofuscinosisSemantic proximity Read

Reduction of Neuroinflammation and Seizures in a Mouse Model of CLN1 Batten Disease using the Small Molecule Enzyme Mimetic, N-Tert-Butyl HydroxylamineSemantic proximity Read

Sex- and region-biased depletion of microglia/macrophages attenuates CLN1 disease in miceSemantic proximity Read

Glial alterations in the glutamatergic and GABAergic signaling pathways in a mouse model of Lafora disease, a severe form of progressive myoclonus epilepsySemantic proximity Read

Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons.Semantic proximity Read

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.Semantic proximity Read

Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis.Semantic proximity Read

Short-term lipopolysaccharide treatment leads to astrocyte activation in LRRK2 G2019S knock-in mice without loss of dopaminergic neuronsSemantic proximity Read

Microglia depletion prior to lipopolysaccharide and paraquat treatment differentially modulates behavioral and neuronal outcomes in wild type and G2019S LRRK2 knock-in mice.Semantic proximity Read

Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice.Semantic proximity Read