Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease)

Sialoadhesin promotes neuroinflammation-related disease progression in two mouse models of CLN disease

(2016) Janos Groh et al.   GLIA

Experimental therapies in the neuronal ceroid lipofuscinoses

(2015) Nicole J. Neverman et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cell biology of the NCL proteins: What they do and don't do

(2015) Jaime Cárcel-Trullols et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

New Zebrafish Models of Neurodegeneration

(2015) Rebeca Martín-Jiménez et al.   Current Neurology and Neuroscience Reports

Modelling inborn errors of metabolism in zebrafish

(2014) Kim Wager et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitophagy and neurodegeneration

(2013) Kim Wager et al.   Autophagy

Genetic basis and phenotypic correlations of the neuronal ceroid lipofusinoses

(2013) Varun Warrier et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

NCL disease mechanisms

(2013) David N. Palmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Use of model organisms for the study of neuronal ceroid lipofuscinosis

(2013) Michael Bond et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

NCL diseases — clinical perspectives

(2013) Angela Schulz et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation

(2013) Fahad Mahmood et al.   BRAIN

Generation and validation of a zebrafish model of EAST (epilepsy, ataxia, sensorineural deafness and tubulopathy) syndrome

(2013) F. Mahmood et al.   Disease Models & Mechanisms

Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway

(2013) Xenia Lojewski et al.   HUMAN MOLECULAR GENETICS

Regional Brain Atrophy in Mouse Models of Neuronal Ceroid Lipofuscinosis

(2013) Thomas G. Kühl et al.   JOURNAL OF CHILD NEUROLOGY

Alterations in ROS Activity and Lysosomal pH Account for Distinct Patterns of Macroautophagy in LINCL and JNCL Fibroblasts

(2013) José Manuel Vidal-Donet et al.   PLoS One

Epilepsy in kcnj10 Morphant Zebrafish Assessed with a Novel Method for Long-Term EEG Recordings

(2013) Anselm A. Zdebik et al.   PLoS One

Human pathology in NCL

(2012) Glenn W. Anderson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mouse models of neuronal ceroid lipofuscinoses: Useful pre-clinical tools to delineate disease pathophysiology and validate therapeutics

(2012) John J. Shacka   BRAIN RESEARCH BULLETIN

Advances in zebrafish chemical screening technologies

(2012) Jonathan R Mathias et al.   Future Medicinal Chemistry

Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease

(2012) Attila D. Kovács et al.   NEUROPHARMACOLOGY

Cardiac involvement in juvenile neuronal ceroid lipofuscinosis (Batten disease)

(2011) J. R. Ostergaard et al.   NEUROLOGY

Knock-Down of Cathepsin D Affects the Retinal Pigment Epithelium, Impairs Swim-Bladder Ontogenesis and Causes Premature Death in Zebrafish

(2011) Carlo Follo et al.   PLoS One

Morpholino artifacts provide pitfalls and reveal a novel role for pro-apoptotic genes in hindbrain boundary development

(2010) Sebastian S. Gerety et al.   DEVELOPMENTAL BIOLOGY

Immunosuppression alters disease severity in juvenile Batten disease mice

(2010) Sabrina S. Seehafer et al.   JOURNAL OF NEUROIMMUNOLOGY

Rapid behavior-based identification of neuroactive small molecules in the zebrafish

(2010) David Kokel et al.   Nature Chemical Biology

Dopaminergic cell damage and vulnerability to MPTP in Pink1 knockdown zebrafish

(2010) Ville Sallinen et al.   NEUROBIOLOGY OF DISEASE

Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease

(2010) Attila D. Kovács et al.   NEUROPHARMACOLOGY

The epidemiology of progressive intellectual and neurological deterioration in childhood

(2009) C. Verity et al.   ARCHIVES OF DISEASE IN CHILDHOOD

Assaying autophagic activity in transgenic GFP-Lc3 and GFP-Gabarap zebrafish embryos

(2009) Congcong He et al.   Autophagy

Autophagy, immunity and human disease

(2009) Robert J Heath et al.   CURRENT OPINION IN GASTROENTEROLOGY

Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease

(2007) Attila D. Kovács et al.   EXPERIMENTAL NEUROLOGY