Saara Tegelberg
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View article: In depth behavioral phenotyping unravels complex motor disturbances in Cstb−/− mouse, a model for progressive myoclonus epilepsy type 1
In depth behavioral phenotyping unravels complex motor disturbances in Cstb−/− mouse, a model for progressive myoclonus epilepsy type 1 Open
Progressive myoclonus epilepsy type 1 (EPM1) is an autosomal recessively inherited childhood–adolescence onset neurodegenerative disease caused by mutations in the cystatin B ( CSTB gene). The key clinical manifestation in EPM1 is progress…
View article: Cystatin B deficiency results in sustained histone H3 tail cleavage in postnatal mouse brain mediated by increased chromatin-associated cathepsin L activity
Cystatin B deficiency results in sustained histone H3 tail cleavage in postnatal mouse brain mediated by increased chromatin-associated cathepsin L activity Open
Cystatin B (CSTB) is a cysteine cathepsin inhibitor whose biallelic loss-of-function mutations in human result in defects in brain development and in neurodegeneration. The physiological function of CSTB is largely unknown, and the mechani…
View article: Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis
Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis Open
View article: Microglial phagocytosis dysfunction in the dentate gyrus is related to local neuronal activity in a genetic model of epilepsy
Microglial phagocytosis dysfunction in the dentate gyrus is related to local neuronal activity in a genetic model of epilepsy Open
Objective Microglial phagocytosis of apoptotic cells is an essential component of the brain regenerative response during neurodegeneration. Whereas it is very efficient in physiological conditions, it is impaired in mouse and human mesial …
View article: Ectopic histone clipping in the mouse model of progressive myoclonus epilepsy
Ectopic histone clipping in the mouse model of progressive myoclonus epilepsy Open
We establish cystatin B (CSTB) as a regulator of histone H3 tail clipping in murine neural progenitor cells (NPCs) and provide evidence suggesting that epigenetic dysregulation contributes to the early pathogenesis in brain disorders assoc…
View article: Microglial phagocytosis dysfunction is related to local neuronal activity in a genetic model of epilepsy
Microglial phagocytosis dysfunction is related to local neuronal activity in a genetic model of epilepsy Open
Microglial phagocytosis of apoptotic cells is an essential component of the brain regenerative response in neurodegenerative diseases. Phagocytosis is very efficient in physiological conditions, as well as during apoptotic challenge induce…
View article: Alternative oxidase‐mediated respiration prevents lethal mitochondrial cardiomyopathy
Alternative oxidase‐mediated respiration prevents lethal mitochondrial cardiomyopathy Open
View article: Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model
Respiratory chain complex III deficiency due to mutated BCS1L: a novel phenotype with encephalomyopathy, partially phenocopied in a Bcs1l mutant mouse model Open
View article: ZNHIT3 is defective in PEHO syndrome, a severe encephalopathy with cerebellar granule neuron loss
ZNHIT3 is defective in PEHO syndrome, a severe encephalopathy with cerebellar granule neuron loss Open
Progressive encephalopathy with oedema, hypsarrhythmia, and optic atrophy (PEHO) syndrome is an early childhood onset, severe autosomal recessive encephalopathy characterized by extreme cerebellar atrophy due to almost total granule neuron…
View article: Brain inflammation is accompanied by peripheral inflammation in Cstb −/− mice, a model for progressive myoclonus epilepsy
Brain inflammation is accompanied by peripheral inflammation in Cstb −/− mice, a model for progressive myoclonus epilepsy Open
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is an autosomal recessively inherited childhood-onset neurodegenerative disorder, characterized by myoclonus, seizures, and ataxia. Mutations in the cystatin B gene (CSTB) u…