Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1 - sprookjes - yvandenbroek - TriplyDB

Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1

Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1

http://www.wikidata.org/entity/Q28582306

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The Role of Stefin B in Neuro-inflammation Hen Egg as an Antioxidant Food Commodity: A Review Shedding new light on neurodegenerative diseases through the mammalian target of rapamycin Macrophage derived cystatin B/cathepsin B in HIV replication and neuropathogenesis Human stefin B role in cell's response to misfolded proteins and autophagy FoxO3a governs early microglial proliferation and employs mitochondrial depolarization with caspase 3, 8, and 9 cleavage during oxidant induced apoptosis.Interaction between oligomers of stefin B and amyloid-beta in vitro and in cells.Diabetes mellitus: channeling care through cellular discovery Altered redox state of monocytes from cryopyrin-associated periodic syndromes causes accelerated IL-1beta secretion.Implications of ER stress, the unfolded protein response, and pro- and anti-apoptotic protein fingerprints in human monocyte-derived dendritic cells treated with alcohol.Cocaine potentiates cathepsin B secretion and neuronal apoptosis from HIV-infected macrophages.Increased oxidative stress and impaired antioxidant response in Lafora disease.Reversal of autophagy dysfunction in the TgCRND8 mouse model of Alzheimer's disease ameliorates amyloid pathologies and memory deficits A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy.A role for stefin B (cystatin B) in inflammation and endotoxemia.Novel avenues of drug discovery and biomarkers for diabetes mellitus.Proteomic analysis of lymphoblastoid cells from Nasu-Hakola patients: a step forward in our understanding of this neurodegenerative disorder.Gene expression alterations in the cerebellum and granule neurons of Cstb(-/-) mouse are associated with early synaptic changes and inflammation.Progressive volume loss and white matter degeneration in cstb-deficient mice: a diffusion tensor and longitudinal volumetry MRI study.Gene-Expression Profiling Suggests Impaired Signaling via the Interferon Pathway in Cstb-/- Microglia Redox processes in neurodegenerative disease involving reactive oxygen species Characterization of a rare Unverricht-Lundborg disease mutation.Role of oxidative stress in refractory epilepsy: evidence in patients and experimental models.Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.The role of initial oligomers in amyloid fibril formation by human stefin B.Neuroprotective effects of flavonoids extracted from licorice on kainate-induced seizure in mice through their antioxidant properties The vitamin nicotinamide: translating nutrition into clinical care.Mechanisms of amyloid fibril formation--focus on domain-swapping.Redox control of NLRP3 inflammasome activation in health and disease.Pore formation by human stefin B in its native and oligomeric states and the consequent amyloid induced toxicity.Progressive myoclonic epilepsy type 1: Report of an Emirati family and literature review.Cystatin B and SOD1: protein–protein interaction and possible relation to neurodegeneration.Brain inflammation is accompanied by peripheral inflammation in Cstb (-/-) mice, a model for progressive myoclonus epilepsy.Putative alternative functions of human stefin B (cystatin B): binding to amyloid-beta, membranes, and copper.Intracellular aggregation of human stefin B: confocal and electron microscopy study.Increased NLRP3-dependent interleukin 1β secretion in patients with familial Mediterranean fever: correlation with MEFV genotype.Human stefin B normal and patho-physiological role: molecular and cellular aspects of amyloid-type aggregation of certain EPM1 mutants.ZNHIT3 is defective in PEHO syndrome, a severe encephalopathy with cerebellar granule neuron loss.Increased expression of stefin B in the nucleus of T98G astrocytoma cells delays caspase activation.Proline Residues as Switches in Conformational Changes Leading to Amyloid Fibril Formation

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Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1

http://www.wikidata.org/entity/Q28582306

description

2009 թուականի Մայիսին հրատարակուած գիտական յօդուած

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2009 թվականի մայիսին հրատարակված գիտական հոդված

@hy

artículu científicu espublizáu en 2009

@ast

im Mai 2009 veröffentlichter wissenschaftlicher Artikel

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scientific journal article

@en

vedecký článok (publikovaný 2009/05/06)

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vědecký článek publikovaný v roce 2009

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wetenschappelijk artikel (gepubliceerd op 2009/05/06)

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наукова стаття, опублікована в травні 2009

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مقالة علمية (نشرت في 6-5-2009)

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name

Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

@ast

Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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type

label

Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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prefLabel

Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

@en

Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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JNEUROSCI.0682-09.2009

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Journal of Neuroscience

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Cystatin B deficiency sensitiz ...... ssive myoclonus epilepsy, EPM1

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Azad Bonni

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Haixiang Su

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Hillel Zukor

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Hyman Schipper

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Maria K. Lehtinen

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Otto Manninen

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Paula Hakala

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Saara Tegelberg

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Tarja Joensuu

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P2860

A conserved MST-FOXO signaling pathway mediates oxidative-stress responses and extends life span

Cathepsin B but not cathepsins L or S contributes to the pathogenesis of Unverricht-Lundborg progressive myoclonus epilepsy (EPM1)

Clinical picture of EPM1-Unverricht-Lundborg disease

Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1)

The cystatins: protein inhibitors of cysteine proteinases

Unstable insertion in the 5' flanking region of the cystatin B gene is the most common mutation in progressive myoclonus epilepsy type 1, EPM1

Progressive ataxia, myoclonic epilepsy and cerebellar apoptosis in cystatin B-deficient mice

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease.

The harlequin mouse mutation downregulates apoptosis-inducing factor.

N-acetylcysteine and Unverricht-Lundborg disease: variable response and possible side effects.

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5910–5915

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10.1523/JNEUROSCI.0682-09.2009

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Anna-Elina Lehesjoki

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24406309

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