| Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis. | Cystatin B-deficiency triggers ectopic histone H3 tail cleavage during neurogenesis.
Daura E, Tegelberg S, Yoshihara M, Jackson C, Simonetti F, Aksentjeff K, Ezer S, Hakala P, Katayama S, Kere J, Lehesjoki AE, Joensuu T. | 01/22/2022 |
| The effects of Cstb duplication on APP/amyloid-beta pathology and cathepsin B activity in a mouse model. | The effects of Cstb duplication on APP/amyloid-β pathology and cathepsin B activity in a mouse model.
Wu Y, Whittaker HT, Noy S, Cleverley K, Brault V, Herault Y, Fisher EMC, Wiseman FK., Free PMC Article | 11/6/2021 |
| Cystatin B is essential for proliferation and interneuron migration in individuals with EPM1 epilepsy. | Cystatin B is essential for proliferation and interneuron migration in individuals with EPM1 epilepsy.
Di Matteo F, Pipicelli F, Kyrousi C, Tovecci I, Penna E, Crispino M, Chambery A, Russo R, Ayo-Martin AC, Giordano M, Hoffmann A, Ciusani E, Canafoglia L, Götz M, Di Giaimo R, Cappello S., Free PMC Article | 09/18/2021 |
| The data of this study show that CXCL13 expression is a hallmark of microglial activation in Cstb (-/-) mice and that the brain inflammation is linked to peripheral inflammatory changes, which might contribute to the disease pathology of progressive myoclonus epilepsy.. | Brain inflammation is accompanied by peripheral inflammation in Cstb (-/-) mice, a model for progressive myoclonus epilepsy.
Okuneva O, Li Z, Körber I, Tegelberg S, Joensuu T, Tian L, Lehesjoki AE., Free PMC Article | 10/7/2017 |
| CSTB has a role in chemotaxis, antigen-presentation, and in immune- and defense response-associated processes by altering JAK-STAT pathway signaling | Gene-Expression Profiling Suggests Impaired Signaling via the Interferon Pathway in Cstb-/- Microglia.
Körber I, Katayama S, Einarsdottir E, Krjutškov K, Hakala P, Kere J, Lehesjoki AE, Joensuu T., Free PMC Article | 07/29/2017 |
| Deletion of the encoding Cstb gene in Npc1-deficient mice resulted in striking deleterious effects, particularly within the cerebellum where diffuse loss of Purkinje cells was observed in young mice. This may be a consequence of damage to lysosomal membranes by reactive oxygen species (ROS), leading to the leakage of lysosomal contents that culminates in apoptosis. | Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.
Chung C, Puthanveetil P, Ory DS, Lieberman AP., Free PMC Article | 01/28/2017 |
| Results suggest a role for CSTB in regulating microglial activation: data link CSTB deficiency to early activation and altered functional properties of microglia, imply presence of both enhanced and suppressed immune response-related microglial functions | Abnormal microglial activation in the Cstb(-/-) mouse, a model for progressive myoclonus epilepsy, EPM1.
Okuneva O, Körber I, Li Z, Tian L, Joensuu T, Kopra O, Lehesjoki AE. | 09/26/2015 |
| the LPS-induced sepsis in StB KO mice is dependent on caspase-11 and mitochondrial reactive oxygen species but is not associated with the lysosomal destabilization and increased cathepsin activity in the cytosol | A role for stefin B (cystatin B) in inflammation and endotoxemia.
Maher K, Jerič Kokelj B, Butinar M, Mikhaylov G, Manček-Keber M, Stoka V, Vasiljeva O, Turk B, Grigoryev SA, Kopitar-Jerala N., Free PMC Article | 01/24/2015 |
| These results indicate an in vivo role for Stfb in protecting cancer cells by promoting their resistance to oxidative stress and to apoptosis induced through the lysosomal pathway. | Stefin B deficiency reduces tumor growth via sensitization of tumor cells to oxidative stress in a breast cancer model.
Butinar M, Prebanda MT, Rajković J, Jerič B, Stoka V, Peters C, Reinheckel T, Krüger A, Turk V, Turk B, Vasiljeva O. | 08/30/2014 |
| Sefin B influences the expression of anti-inflammatory IL-10 in response to TLR4 agonists. | Decreased IL-10 expression in stefin B-deficient macrophages is regulated by the MAP kinase and STAT-3 signaling pathways.
Maher K, Završnik J, Jerič-Kokelj B, Vasiljeva O, Turk B, Kopitar-Jerala N. | 04/19/2014 |
| An increase in Cstb does not induce any spontaneous epileptic activity. | Characterization of PTZ-induced seizure susceptibility in a down syndrome mouse model that overexpresses CSTB.
Brault V, Martin B, Costet N, Bizot JC, Hérault Y., Free PMC Article | 04/14/2012 |
| Pathologic events in the CSTB-deficient brain highlight the potential role of glial activation at the initial stages of progressive myoclonic epilepsy type 1. | Early microglial activation precedes neuronal loss in the brain of the Cstb-/- mouse model of progressive myoclonus epilepsy, EPM1.
Tegelberg S, Kopra O, Joensuu T, Cooper JD, Lehesjoki AE. | 02/18/2012 |
| this study suggesting that the Cstb-Prmt2 region is not playing a major role in locomotor and cognitive deficits found in mice model of Down syndrome. | The telomeric part of the human chromosome 21 from Cstb to Prmt2 is not necessary for the locomotor and short-term memory deficits observed in the Tc1 mouse model of Down syndrome.
Duchon A, Pothion S, Brault V, Sharp AJ, Tybulewicz VL, Fisher EM, Herault Y., Free PMC Article | 04/23/2011 |
| Data show that cystatin C effectively rescues cystatin B loss-of-function mutation, facilitating the reversal of pathophysiological changes and suggesting a novel therapeutic intervention for patients with neurodegenerative disorders. | Cystatin C rescues degenerating neurons in a cystatin B-knockout mouse model of progressive myoclonus epilepsy.
Kaur G, Mohan P, Pawlik M, DeRosa S, Fajiculay J, Che S, Grubb A, Ginsberg SD, Nixon RA, Levy E., Free PMC Article | 03/26/2011 |
| Stefin B interacts with histones and cathepsin L in the nucleus | Stefin B interacts with histones and cathepsin L in the nucleus.
Čeru S, Konjar Š, Maher K, Repnik U, Križaj I, Benčina M, Renko M, Nepveu A, Žerovnik E, Turk B, Kopitar-Jerala N., Free PMC Article | 05/3/2010 |
| This study identifies a novel function for Cystatin B in defending cerebellar granule neurons from oxidative stress. | Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1.
Lehtinen MK, Tegelberg S, Schipper H, Su H, Zukor H, Manninen O, Kopra O, Joensuu T, Hakala P, Bonni A, Lehesjoki AE., Free PMC Article | 01/21/2010 |
| Increased cleavage of MARCKS in brain and macrophages, but not in liver and kidney extracts of stefin B-deficient mice compared to wild-type mice, was observed. | Cleavage of the myristoylated alanine-rich C kinase substrate (MARCKS) by cysteine cathepsins in cells and tissues of stefin B-deficient mice.
Kopitar-Jerala N, Turk B. | 01/21/2010 |
| These findings indicate that neuronal atrophy is an important consequence of cystatin-B deficiency independent of seizure events, suggesting a physiological role for this protein in the maintenance of normal neuronal structure. | Neuropathological changes in a mouse model of progressive myoclonus epilepsy: cystatin B deficiency and Unverricht-Lundborg disease.
Shannon P, Pennacchio LA, Houseweart MK, Minassian BA, Myers RM. | 01/21/2010 |
| greater degree of neuronal damage was observed in CSTB-deficient than in WT mice after seizures of identical grade, indicating increased susceptibility to seizure-induced cell death | A pathogenetic hypothesis of Unverricht-Lundborg disease onset and progression.
Franceschetti S, Sancini G, Buzzi A, Zucchini S, Paradiso B, Magnaghi G, Frassoni C, Chikhladze M, Avanzini G, Simonato M. | 01/21/2010 |