Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)

Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)

The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited neurodegenerative diseases characterized by gross brain atrophy, blindness, and intracellular accumulation of lysosome-derived storage bodies. A CLN6 form in sheep is studied as a large animal model of the human diseases....

Full description

Bibliographic Details
Main Authors:	Manfred J. Oswald, David N. Palmer, Graham W. Kay, Stephen J.A. Shemilt, Payam Rezaie, Jonathan D. Cooper
Format:	Article
Language:	English
Published:	Elsevier 2005-10-01
Series:	Neurobiology of Disease
Subjects:	Astrocytosis Batten disease Brain macrophages Fluorescent Lysosomal storage disease Microglia
Online Access:	http://www.sciencedirect.com/science/article/pii/S0969996105000446

Similar Items

Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons
by: Lotta Parviainen, et al.
Published: (2017-10-01)

in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels
by: Alamin Mohammed, et al.
Published: (2017-07-01)

CLN6 Variant of Late Infantile Neuronal Ceroid Lipofuscinosis Caused by a Homozygous Mutation: Case Report in Colombia
by: Daniel Eduardo Manrique Hernandez, et al.
Published: (2023-01-01)

Neuronal Ceroid Lipofuscinosis in a Domestic Cat Associated with a DNA Sequence Variant That Creates a Premature Stop Codon in CLN6
by: Martin L. Katz, et al.
Published: (2020-08-01)

Age-dependent alterations in neuronal activity in the hippocampus and visual cortex in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis (CLN3)
by: Maria Burkovetskaya, et al.
Published: (2017-04-01)

Neuronal Ceroid Lipofuscinosis Type 6 (CLN6) clinical findings and molecular diagnosis: Costa Rica’s experience
by: R. Badilla-Porras, et al.
Published: (2022-01-01)

A Novel CLN6 Variant Associated With Juvenile Neuronal Ceroid Lipofuscinosis in Patients With Absence of Visual Loss as a Presenting Feature
by: Paschalis Nicolaou, et al.
Published: (2021-11-01)

Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis
by: Benedikt Grünewald, et al.
Published: (2017-11-01)

Cardiac magnetic resonance findings in neuronal ceroid lipofuscinosis: A case report
by: Giancarlo Todiere, et al.
Published: (2022-08-01)

The Challenges of Living with and Caring for a Child or Children Affected by Neuronal Ceroid Lipofuscinosis Type 2 Disease: In-Depth Family Surveys in the United Kingdom and Germany
by: Angela Schulz, et al.

Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis
by: Jenny Lange, et al.
Published: (2018-08-01)

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis
by: Catherine Kielar, et al.
Published: (2007-01-01)

Converging links between adult-onset neurodegenerative Alzheimer’s disease and early life neurodegenerative neuronal ceroid lipofuscinosis?
by: Marcel Klein, et al.
Published: (2023-01-01)

Classic and Atypical Late Infantile Neuronal Ceroid Lipofuscinosis in Latin America: Clinical and Genetic Aspects, and Treatment Outcome with Cerliponase Alfa
by: Norberto Guelbert, et al.
Published: (2024-03-01)

Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum
by: Jonathan E. Phillips, et al.
Published: (2015-02-01)

Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice
by: Tuhin Virmani, et al.
Published: (2005-11-01)

Modelling of Neuronal Ceroid Lipofuscinosis Type 2 in <i>Dictyostelium discoideum</i> Suggests That Cytopathological Outcomes Result from Altered TOR Signalling
by: Paige K. Smith, et al.
Published: (2019-05-01)

A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>>>A) leading to excision of exon 3
by: Tony Frugier, et al.
Published: (2008-02-01)

Juvenile neuronal ceroid-lipofuscinosis: clinical and molecular investigation in a large family in Brazil
by: Eugênia Ribeiro Valadares, et al.
Published: (2011-02-01)

Emerging new roles of the lysosome and neuronal ceroid lipofuscinoses
by: Anil B. Mukherjee, et al.
Published: (2019-01-01)

Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa
by: J. Schaefers, et al.
Published: (2021-05-01)

Paroxysmal sympathetic hyperactivity following status epilepticus in a 22-year-old with Juvenile Neuronal Ceroid Lipofuscinosis: A case report
by: Molly D. Himmelrich, et al.
Published: (2021-01-01)

The CLN3 gene and protein: What we know
by: Myriam Mirza, et al.
Published: (2019-12-01)

Autophagy in the Neuronal Ceroid Lipofuscinoses (Batten Disease)
by: William D. Kim, et al.
Published: (2022-02-01)

Etiology of anxious and fearful behavior in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)
by: John R. Ostergaard
Published: (2023-04-01)

An Unusual Presentation of Neuronal Ceroid Lipofuscinosis With CLN6 Mutation
by: Shahin Koohmanaee, et al.
Published: (2023-01-01)

CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)
by: S. L. Eaton, et al.
Published: (2019-07-01)

Phenotypic variant of CLN3 mutation
by: Avinash Honasoge, et al.
Published: (2022-09-01)

Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis.
by: Katja M Kanninen, et al.
Published: (2013-01-01)

A Case Report on the Challenging Diagnosis of Neuronal Ceroid Lipofuscinosis Type 2 (CLN2)
by: Andrea Nunes, et al.
Published: (2020-12-01)

Aggregation chimeras provide evidence of in vivo intercellular correction in ovine CLN6 neuronal ceroid lipofuscinosis (Batten disease).
by: Lucy Anne Barry, et al.
Published: (2022-01-01)

Aggregation chimeras provide evidence of in vivo intercellular correction in ovine CLN6 neuronal ceroid lipofuscinosis (Batten disease)
by: Lucy Anne Barry, et al.
Published: (2022-01-01)

Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3−/− Mice
by: Marta A. Tarczyluk-Wells, et al.
Published: (2019-09-01)

Molecular networking in the neuronal ceroid lipofuscinoses: insights from mammalian models and the social amoeba Dictyostelium discoideum
by: Robert J. Huber
Published: (2020-05-01)

The parent and family impact of CLN3 disease: an observational survey-based study
by: Angela Schulz, et al.
Published: (2024-03-01)

Neuronal Ceroid Lipofuscinoses: Connecting Calcium Signalling through Calmodulin
by: Sabateeshan Mathavarajah, et al.
Published: (2018-10-01)

The conserved cellular roles of CLN proteins: Novel insights from Dictyostelium discoideum
by: Adam A.N. Remtulla, et al.
Published: (2023-06-01)

Experimental Therapeutic Approaches for the Treatment of Retinal Pathology in Neuronal Ceroid Lipofuscinoses
by: Udo Bartsch, et al.
Published: (2022-04-01)

Extracellular Vesicles as Drug Carriers for Enzyme Replacement Therapy to Treat CLN2 Batten Disease: Optimization of Drug Administration Routes
by: Matthew J. Haney, et al.
Published: (2020-05-01)

Neuronal ceroid lipofuscinosis: A case report
by: Gopen Kumar Kundu, et al.
Published: (2023-09-01)