Cargando…

Comprehensive functional characterization of murine infantile Batten disease including Parkinson-like behavior and dopaminergic markers

Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten disease) is a neurodegenerative lysosomal storage disease caused by a deficiency in palmitoyl protein thioesterase-1 (PPT1). The PPT1-deficient mouse (Cln1(−/−)) is a useful phenocopy of human INCL. Cln1(−/−) mice display retinal dysfu...

Descripción completa

Detalles Bibliográficos
Autores principales:	Dearborn, Joshua T., Harmon, Steven K., Fowler, Stephen C., O’Malley, Karen L., Taylor, George T., Sands, Mark S., Wozniak, David F.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group 2015
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4523849/ https://www.ncbi.nlm.nih.gov/pubmed/26238334 http://dx.doi.org/10.1038/srep12752

Ejemplares similares

Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy
por: Reddy, Adarsh S., et al.
Publicado: (2012)

The Parkinsonian mimetic, 6-OHDA, impairs axonal transport in dopaminergic axons
por: Lu, Xi, et al.
Publicado: (2014)

Cellular models of Batten disease
por: Minnis, Christopher J., et al.
Publicado: (2020)

Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease
por: Kielar, Catherine, et al.
Publicado: (2009)

Altered protein secretion in Batten disease
por: Huber, Robert J.
Publicado: (2021)

Batten disease: lipid transport goes awry
Publicado: (2012)

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

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)

Wld(S )but not Nmnat1 protects dopaminergic neurites from MPP(+ )neurotoxicity
por: Antenor-Dorsey, Jo Ann V, et al.
Publicado: (2012)

pH-dependent regulation of Batten disease protein
Publicado: (2011)

Clinical aspects of neuroregression: our experience on batten disease
por: Kamate, Mahesh
Publicado: (2014)

The converging roles of Batten disease proteins in neurodegeneration and cancer
por: Yap, Shyong Quan, et al.
Publicado: (2021)

Batten's Disease: A Seizure Disorder's Battle for Diagnosis
por: Dev, Nishanth, et al.
Publicado: (2021)

The Role of Axonopathy in Parkinson's Disease
por: O'Malley, Karen L.
Publicado: (2010)

Optimising preclinical mouse models: lesson from Batten disease
Publicado: (2015)

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

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

The Batten disease gene CLN3 is required for the response to oxidative stress
por: Tuxworth, Richard I., et al.
Publicado: (2011)

Efficacy of phosphodiesterase‐4 inhibitors in juvenile Batten disease (CLN3)
por: Aldrich, Amy, et al.
Publicado: (2016)

Modulating membrane fluidity corrects Batten disease phenotypes in vitro and in vivo
por: Schultz, Mark L., et al.
Publicado: (2018)

Evaluation of the Results of Septum Extension Batten Graft in Patients Referred for Septorhinoplasty
por: Hosnani, Hamidreza, et al.
Publicado: (2023)

Parkinson's disease and dopaminergic neurons
Publicado: (2012)

Conditional knockout of UBC13 produces disturbances in gait and spontaneous locomotion and exploration in mice
por: Wozniak, David F., et al.
Publicado: (2019)

Deficiency in endocannabinoid synthase DAGLB contributes to early onset Parkinsonism and murine nigral dopaminergic neuron dysfunction
por: Liu, Zhenhua, et al.
Publicado: (2022)

Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease)
por: Wager, Kim, et al.
Publicado: (2016)

Discovery of a CLN7 model of Batten disease in non-human primates
por: McBride, Jodi L., et al.
Publicado: (2018)

Current Insights in Elucidation of Possible Molecular Mechanisms of the Juvenile Form of Batten Disease
por: Shematorova, Elena K., et al.
Publicado: (2020)

Gene Therapy Corrects Brain and Behavioral Pathologies in CLN6-Batten Disease
por: Cain, Jacob T., et al.
Publicado: (2019)

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

Recognizing differentiating clinical signs of CLN3 disease (Batten disease) at presentation
por: Kuper, Willemijn F. E., et al.
Publicado: (2020)

Application of Anticonvulsants, Antiepileptic Drugs, and Vitamin C in the Treatment and Analysis of Batten Disease
por: Reddy, Shreya, et al.
Publicado: (2022)

Acidified drinking water improves motor function, prevents tremors and changes disease trajectory in Cln2(R207X) mice, a model of late infantile Batten disease
por: Kovács, Attila D., et al.
Publicado: (2023)

Parkinson’s Disease, the Dopaminergic Neuron and Gammahydroxybutyrate
por: Mamelak, Mortimer
Publicado: (2018)

Curso de tratamiento infantil/
por: Johnson, James H. (James Harmon), 1943-
Publicado: (1986)

pH-dependent localization of Btn1p in the yeast model for Batten disease
por: Wolfe, Devin M., et al.
Publicado: (2011)

Osmotic Stress Changes the Expression and Subcellular Localization of the Batten Disease Protein CLN3
por: Getty, Amanda, et al.
Publicado: (2013)

Internal Nasal Valve Incompetence Is Effectively Treated Using Batten Graft Functional Rhinoplasty
por: Bewick, J. C., et al.
Publicado: (2013)

Searching for novel biomarkers using a mouse model of CLN3-Batten disease
por: Timm, Derek, et al.
Publicado: (2018)

Tracking sex-dependent differences in a mouse model of CLN6-Batten disease
por: Poppens, McKayla J., et al.
Publicado: (2019)

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

Cannot write session to /tmp/vufind_sessions/sess_atavu6ssvhp1ehd9ntm551kdcg