[HTML][HTML] Quantitative changes in the mitochondrial proteome of cerebellar synaptosomes from preclinical cystatin B-deficient mice
K Gorski, A Spoljaric, TA Nyman, K Kaila… - Frontiers in molecular …, 2020 - frontiersin.org
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is a
neurodegenerative disorder caused by loss-of-function mutations in the cystatin B (CSTB) …
neurodegenerative disorder caused by loss-of-function mutations in the cystatin B (CSTB) …
[HTML][HTML] Progressive mitochondrial dysfunction in cerebellar synaptosomes of cystatin B-deficient mice
K Gorski, CB Jackson, TA Nyman, V Rezov… - Frontiers in Molecular …, 2023 - frontiersin.org
The involvement of mitochondrial dysfunction in cystatin B (CSTB) deficiency has been
suggested, but its role in the onset of neurodegeneration, myoclonus, and ataxia in the …
suggested, but its role in the onset of neurodegeneration, myoclonus, and ataxia in the …
Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1
MK Lehtinen, S Tegelberg, H Schipper… - Journal of …, 2009 - Soc Neuroscience
The progressive myoclonus epilepsies, featuring the triad of myoclonus, seizures, and
ataxia, comprise a large group of inherited neurodegenerative diseases that remain poorly …
ataxia, comprise a large group of inherited neurodegenerative diseases that remain poorly …
Gene Expression Alterations in the Cerebellum and Granule Neurons of Cstb−/− Mouse Are Associated with Early Synaptic Changes and Inflammation
T Joensuu, S Tegelberg, E Reinmaa, M Segerstråle… - PloS one, 2014 - journals.plos.org
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is an autosomal
recessively inherited neurodegenerative disease, manifesting with myoclonus, seizures and …
recessively inherited neurodegenerative disease, manifesting with myoclonus, seizures and …
Abnormal microglial activation in the Cstb−/− mouse, a model for progressive myoclonus epilepsy, EPM1
Progressive myoclonus epilepsy of Unverricht–Lundborg type (EPM1) is an autosomal‐
recessively inherited neurodegenerative disorder characterized by severely incapacitating …
recessively inherited neurodegenerative disorder characterized by severely incapacitating …
Early Microglial Activation Precedes Neuronal Loss in the Brain of the Cstb−/− Mouse Model of Progressive Myoclonus Epilepsy, EPM1
S Tegelberg, O Kopra, T Joensuu… - … of Neuropathology & …, 2012 - academic.oup.com
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1) is a hereditary
neurodegenerative disorder caused by mutations in the cystatin B (CSTB) gene encoding an …
neurodegenerative disorder caused by mutations in the cystatin B (CSTB) gene encoding an …
[HTML][HTML] In depth behavioral phenotyping unravels complex motor disturbances in Cstb−/− mouse, a model for progressive myoclonus epilepsy type 1
E Pollari, S Tegelberg, H Björklund… - Frontiers in …, 2023 - ncbi.nlm.nih.gov
Progressive myoclonus epilepsy type 1 (EPM1) is an autosomal recessively inherited
childhood–adolescence onset neurodegenerative disease caused by mutations in the …
childhood–adolescence onset neurodegenerative disease caused by mutations in the …
Gene-Expression Profiling Suggests Impaired Signaling via the Interferon Pathway in Cstb-/- Microglia
I Körber, S Katayama, E Einarsdottir, K Krjutškov… - PloS one, 2016 - journals.plos.org
Progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1, OMIM254800) is an
autosomal recessive neurodegenerative disorder characterized by stimulus-sensitive and …
autosomal recessive neurodegenerative disorder characterized by stimulus-sensitive and …
Cystatin B is essential for proliferation and interneuron migration in individuals with EPM 1 epilepsy
F Di Matteo, F Pipicelli, C Kyrousi, I Tovecci… - EMBO molecular …, 2020 - embopress.org
Progressive myoclonus epilepsy (PME) of Unverricht–Lundborg type (EPM 1) is an
autosomal recessive neurodegenerative disorder with the highest incidence of PME …
autosomal recessive neurodegenerative disorder with the highest incidence of PME …
The Roles of Cystatin B in the Brain and Pathophysiological Mechanisms of Progressive Myoclonic Epilepsy Type 1
S Singh, RH Hämäläinen - Cells, 2024 - mdpi.com
Progressive myoclonic epilepsy type 1 (EPM1) is an autosomal recessive disorder, also
known as Unverricht–Lundborg disease (ULD). EPM1 patients suffer from photo-sensitive …
known as Unverricht–Lundborg disease (ULD). EPM1 patients suffer from photo-sensitive …