Article

Lack of Cathepsin D in the Central Nervous System Results in Microglia and Astrocyte Activation and the Accumulation of Proteinopathy-related Proteins

2021-11-30

Abstract excerpt

Neuronal ceroid lipofuscinosis is one of many neurodegenerative storage diseases characterized by excessive accumulation of lipofuscins. CLN10 disease, an early infantile neuronal ceroid lipofuscinosis, is associated with a gene that encodes cathepsin D (CtsD), one of the major lysosomal proteases. Whole body CtsD-knockout mice show neurodegenerative phenotypes with the accumulation of lipofuscins in the brain and...

Topics

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

Identifiers and source

Literature Corpus work: 003a981c-6fe9-528b-a909-2041b2c75cbb
DOI: 10.21203/rs.3.rs-1080356/v1

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Lack of Cathepsin D in the Central Nervous System Results in Microglia and Astrocyte Activation and the Accumulation of Proteinopathy-related ProteinsDOI 10.21203/rs.3.rs-1080356/v1Read

Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons.Semantic proximity Read

Neuronal Ceroid Lipofuscinosis—Concepts, Classification, and Avenues for TherapySemantic proximity Read

Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disorders.Semantic proximity Read

Murine Cathepsin F Deficiency Causes Neuronal Lipofuscinosis and Late-Onset Neurological DiseaseSemantic proximity Read

A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration.Semantic proximity Read

Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis.Semantic proximity Read

Altered expression and distribution of cathepsins in neuronopathic forms of Gaucher disease and in other sphingolipidosesSemantic proximity Read

A mouse mutant deficient in both neuronal ceroid lipofuscinosis-associated proteins CLN3 and TPP1.Semantic proximity Read

Cathepsin D deficiency causes juvenile-onset ataxia and distinctive muscle pathologySemantic proximity Read

A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.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

Novel Cln8 p.R24G mouse line replicates major clinical features of Northern epilepsySemantic proximity Read