Therapeutic landscape for Batten disease: current treatments and future prospects
TB Johnson, JT Cain, KA White… - Nature Reviews …, 2019 - nature.com
Batten disease (also known as neuronal ceroid lipofuscinoses) constitutes a family of
devastating lysosomal storage disorders that collectively represent the most common …
devastating lysosomal storage disorders that collectively represent the most common …
[HTML][HTML] Neuronal ceroid lipofuscinoses
A Jalanko, T Braulke - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2009 - Elsevier
The neuronal ceroid lipofuscinoses (NCL) are severe neurodegenerative lysosomal storage
disorders of childhood, characterized by accumulation of autofluorescent ceroid …
disorders of childhood, characterized by accumulation of autofluorescent ceroid …
Chronic stress followed by social isolation promotes depressive-like behaviour, alters microglial and astrocyte biology and reduces hippocampal neurogenesis in …
Unpredictable chronic mild stress (UCMS) is one of the most commonly used, robust and
translatable models for studying the neurobiological basis of major depression. Although the …
translatable models for studying the neurobiological basis of major depression. Although the …
The type of stress matters: repeated injection and permanent social isolation stress in male mice have a differential effect on anxiety-and depressive-like behaviours …
A Du Preez, T Law, D Onorato, YM Lim, P Eiben… - Translational …, 2020 - nature.com
Chronic stress can alter the immune system, adult hippocampal neurogenesis and induce
anxiety-and depressive-like behaviour in rodents. However, previous studies have not …
anxiety-and depressive-like behaviour in rodents. However, previous studies have not …
The neuronal ceroid-lipofuscinoses (Batten disease)
SE Mole, A Schulz - Rosenberg's Molecular and Genetic Basis of …, 2025 - Elsevier
The neuronal ceroid-lipofuscinoses (NCLs), collectively also called Batten disease,
constitute one of the most common groups of inherited neurodegenerative disorders in …
constitute one of the most common groups of inherited neurodegenerative disorders in …
Uncovering molecular biomarkers that correlate cognitive decline with the changes of hippocampus' gene expression profiles in Alzheimer's disease
Background Alzheimer's disease (AD) is characterized by a neurodegenerative progression
that alters cognition. On a phenotypical level, cognition is evaluated by means of the …
that alters cognition. On a phenotypical level, cognition is evaluated by means of the …
[HTML][HTML] Pathomechanisms in the neuronal ceroid lipofuscinoses
The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative
lysosomal storage disorders (LSDs), traditionally grouped together based on shared clinical …
lysosomal storage disorders (LSDs), traditionally grouped together based on shared clinical …
Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis
C Kielar, L Maddox, E Bible, CC Pontikis… - Neurobiology of …, 2007 - Elsevier
Infantile neuronal ceroid lipofuscinosis (INCL) is caused by deficiency of the lysosomal
enzyme, palmitoyl protein thioesterase 1 (PPT1). We have investigated the onset and …
enzyme, palmitoyl protein thioesterase 1 (PPT1). We have investigated the onset and …
Late onset neurodegeneration in the Cln3−/− mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation
CC Pontikis, CV Cella, N Parihar, MJ Lim… - Brain research, 2004 - Elsevier
Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human
disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in …
disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in …
[HTML][HTML] NCL disease mechanisms
Despite the identification of a large number of disease-causing genes in recent years, it is
still unclear what disease mechanisms operate in the neuronal ceroid lipofuscinoses (NCLs …
still unclear what disease mechanisms operate in the neuronal ceroid lipofuscinoses (NCLs …