Cargando…

Aggregation chimeras provide evidence of in vivo intercellular correction in ovine CLN6 neuronal ceroid lipofuscinosis (Batten disease)

The neuronal ceroid lipofuscinoses (NCLs; Batten disease) are fatal, mainly childhood, inherited neurodegenerative lysosomal storage diseases. Sheep affected with a CLN6 form display progressive regionally defined glial activation and subsequent neurodegeneration, indicating that neuroinflammation m...

Descripción completa

Detalles Bibliográficos
Autores principales:	Barry, Lucy Anne, Kay, Graham William, Mitchell, Nadia Lesley, Murray, Samantha Jane, Jay, Nigel P., Palmer, David Norris
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2022
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9000108/ https://www.ncbi.nlm.nih.gov/pubmed/35404973 http://dx.doi.org/10.1371/journal.pone.0261544

Ejemplares similares

An ERG and OCT study of neuronal ceroid lipofuscinosis CLN2 Battens retinopathy
por: Thompson, Dorothy A., et al.
Publicado: (2021)

Correction: An ERG and OCT study of neuronal ceroid lipofuscinosis CLN2 Battens retinopathy
por: Thompson, Dorothy A., et al.
Publicado: (2021)

Progressive MRI brain volume changes in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinosis
por: Murray, Samantha J, et al.
Publicado: (2023)

Juvenile neuronal ceroid lipofuscinosis (Batten disease): current insights
por: Ostergaard, John R
Publicado: (2016)

Untargeted Metabolite Profiling of Cerebrospinal Fluid Uncovers Biomarkers for Severity of Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2, Batten Disease)
por: Sindelar, Miriam, et al.
Publicado: (2018)

CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)
por: Eaton, S. L., et al.
Publicado: (2019)

Chronic oral administration of minocycline to sheep with ovine CLN6 neuronal ceroid lipofuscinosis maintains pharmacological concentrations in the brain but does not suppress neuroinflammation or disease progression
por: Kay, Graham W, et al.
Publicado: (2013)

Novel CLN1 mutation with atypical juvenile neuronal ceroid lipofuscinosis
por: Khan, Arif, et al.
Publicado: (2013)

in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels
por: Mohammed, Alamin, et al.
Publicado: (2017)

A lysosomal enigma CLN5 and its significance in understanding neuronal ceroid lipofuscinosis
por: Basak, I., et al.
Publicado: (2021)

Electroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses
por: Russell, Katharina N., et al.
Publicado: (2021)

Aberrant upregulation of the glycolytic enzyme PFKFB3 in CLN7 neuronal ceroid lipofuscinosis
por: Lopez-Fabuel, Irene, et al.
Publicado: (2022)

A Missense Mutation in Canine CLN6 in an Australian Shepherd with Neuronal Ceroid Lipofuscinosis
por: Katz, Martin L., et al.
Publicado: (2011)

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice
por: Blom, Tea, et al.
Publicado: (2013)

Etiology of anxious and fearful behavior in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)
por: Ostergaard, John R.
Publicado: (2023)

Increased Zinc and Manganese in Parallel with Neurodegeneration, Synaptic Protein Changes and Activation of Akt/GSK3 Signaling in Ovine CLN6 Neuronal Ceroid Lipofuscinosis
por: Kanninen, Katja M., et al.
Publicado: (2013)

Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins
por: Lyly, Annina, et al.
Publicado: (2009)

The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development
por: Connolly, Kyle J., et al.
Publicado: (2019)

Characterization of neurological disease progression in a canine model of CLN5 neuronal ceroid lipofuscinosis
por: Meiman, Elizabeth J., et al.
Publicado: (2022)

Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3(Δex1-6) mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease)
por: Wavre-Shapton, Silène T., et al.
Publicado: (2015)

Value of genetic testing for pediatric epilepsy: Driving earlier diagnosis of ceroid lipofuscinosis type 2 Batten disease
por: Leal‐Pardinas, Fernanda, et al.
Publicado: (2022)

Natural history of retinal degeneration in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses
por: Murray, S. J., et al.
Publicado: (2022)

Association between CLN3 (Neuronal Ceroid Lipofuscinosis, CLN3 Type) Gene Expression and Clinical Characteristics of Breast Cancer Patients
por: Makoukji, Joelle, et al.
Publicado: (2015)

Assessing the integrity of auditory sensory memory processing in CLN3 disease (Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)): An auditory evoked potential study of the duration-evoked mismatch negativity (MMN)
por: Brima, Tufikameni, et al.
Publicado: (2023)

Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis
por: Kanninen, Katja M., et al.
Publicado: (2013)

Novel likely disease-causing CLN5 variants identified in Pakistani patients with neuronal ceroid lipofuscinosis
por: Azad, Beenish, et al.
Publicado: (2020)

Functional Analysis of a Novel CLN5 Mutation Identified in a Patient With Neuronal Ceroid Lipofuscinosis
por: Luo, Sukun, et al.
Publicado: (2020)

Rare adult neuronal ceroid lipofuscinosis associated with CLN6 gene mutations: A case report
por: Wang, Xue-Qiang, et al.
Publicado: (2023)

Data on characterizing the gene expression patterns of neuronal ceroid lipofuscinosis genes: CLN1, CLN2, CLN3, CLN5 and their association to interneuron and neurotransmission markers: Parvalbumin and Somatostatin
por: Minye, Helena M., et al.
Publicado: (2016)

Mutation update: Review of TPP1 gene variants associated with neuronal ceroid lipofuscinosis CLN2 disease
por: Gardner, Emily, et al.
Publicado: (2019)

A Drosophila model of neuronal ceroid lipofuscinosis CLN4 reveals a hypermorphic gain of function mechanism
por: Imler, Elliot, et al.
Publicado: (2019)

CLN8 Mutations Presenting with a Phenotypic Continuum of Neuronal Ceroid Lipofuscinosis—Literature Review and Case Report
por: Badura-Stronka, Magdalena, et al.
Publicado: (2021)

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

Pacemaker Implantation in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3)–A Long-Term Follow-Up Study
por: Handrup, Mette Møller, et al.
Publicado: (2022)

Autophagy in the Neuronal Ceroid Lipofuscinoses (Batten Disease)
por: Kim, William D., et al.
Publicado: (2022)

Autophagy–lysosome pathway alterations and alpha-synuclein up-regulation in the subtype of neuronal ceroid lipofuscinosis, CLN5 disease
por: Adams, Jessie, et al.
Publicado: (2019)

Neuronal ceroid lipofuscinosis in a German Shorthaired Pointer associated with a previously reported CLN8 nonsense variant
por: Guo, Juyuan, et al.
Publicado: (2019)

Treatment of non-epileptic episodes of anxious, fearful behavior in adolescent juvenile neuronal ceroid lipofuscinosis (CLN3 disease)
por: Ostergaard, John R.
Publicado: (2023)

MRI findings in neuronal ceroid lipofuscinosis
por: Crain, Anna M., et al.
Publicado: (2020)

Molecular neuropathology of the synapse in sheep with CLN5 Batten disease
por: Amorim, Inês S., et al.
Publicado: (2015)

Cannot write session to /tmp/vufind_sessions/sess_bf4o5lmjno5an2gp1ua792e9et