A5048225695- Amyotrophic Lateral Sclerosis Research
- Parkinson's Disease Mechanisms and Treatments
- Alzheimer's disease research and treatments
- Neurogenetic and Muscular Disorders Research
- Cholinesterase and Neurodegenerative Diseases
- Neuroscience and Neuropharmacology Research
- Nuclear Receptors and Signaling
- RNA Research and Splicing
- Ubiquitin and proteasome pathways
- Nerve injury and regeneration
- Genetic Neurodegenerative Diseases
- Computational Drug Discovery Methods
- Biochemical Acid Research Studies
- Mitochondrial Function and Pathology
- Synthesis and bioactivity of alkaloids
- Neurological disorders and treatments
- COVID-19 Clinical Research Studies
- GDF15 and Related Biomarkers
- SARS-CoV-2 and COVID-19 Research
- Heat shock proteins research
- 3D Printing in Biomedical Research
- Sphingolipid Metabolism and Signaling
- Neurological Disorders and Treatments
- Prion Diseases and Protein Misfolding
- 14-3-3 protein interactions
Institute of Physiologically Active Compounds
2015-2024
Institute of Molecular and Cell Biology
2024
Cardiff University
2009-2020
Russian Academy of Sciences
2008-2015
Washington State University
2009
Lomonosov Moscow State University
2005
Pacific Northwest National Laboratory
2002-2003
Dysfunction of two structurally and functionally related proteins, FUS TAR DNA-binding protein 43 kDa (TDP-43), implicated in crucial steps cellular RNA metabolism can cause amyotrophic lateral sclerosis (ALS) certain other neurodegenerative diseases. The proteins are intrinsically aggregate-prone form non-amyloid inclusions the affected nervous tissues, but role these proteinaceous aggregates disease onset progression is still uncertain. To address this question, we designed a variant FUS,...
Cognitive malfunction, synaptic dysfunction, and disconnections in neural networks are core deficits Alzheimer’s disease (AD). 5xFAD mice, a transgenic model of AD, characterized by an enhanced level amyloid-β abnormal neurotransmission. The dopaminergic (DA) system has been shown to be involved transformations neuronal plasticity; however, its role functional network changes familial AD still remains unclear. In non-transgenic freely moving electroencephalograms (EEGs) were simultaneously...
The etiology and pathogenesis of Parkinson's disease (PD) are tightly linked to the gain-of-function α-synuclein. However, gradual accumulation α-synuclein aggregates in dopaminergic neurons substantia nigra pars compacta (SNpc) leads depletion functional pool soluble α-synuclein, therefore, creates loss-of-function conditions, particularly presynaptic terminals these neurons. Studies how this late-onset a protein involved many important steps neurotransmission contributes PD progression...
Intermediates and final products of protein aggregation play crucial role in the development degenerative changes a number neurological diseases. Pathological is currently regarded as one most promising therapeutic targets for treatment these Transgenic mouse models proteinopathies are an effective tool screening validation compounds, which can selectively affect metabolism aggregate-prone proteins. In this study, we assessed effects dimebon, compound with known neuroprotective properties,...
The Y-box binding protein 1 (YB-1) is a member of the family DNA- and RNA proteins. It involved in wide variety DNA/RNA-dependent events including cell proliferation differentiation, stress response, malignant transformation. Previously, YB-1 was detected neurons neocortex hippocampus, but its precise role brain remains undefined. Here we show that subchronic intranasal injections recombinant YB-1, as well fragment YB-11−219, suppress impairment spatial memory olfactory bulbectomized (OBX)...
Abstract Pathological modification of α-synuclein is believed to be an important event in pathogenesis Parkinson’s disease and several other neurodegenerative diseases. In normal cells this protein has been linked many intracellular processes pathways. However, neither function neuronal certain types nor its exact role the well understood, which largely due limitations animal models used for studying protein. We produced validated novel mouse lines manipulating expression endogenous Snca...
Abstract Dysfunction of the RNA-binding protein (RBP) FUS implicated in RNA metabolism can cause amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. Mutations affecting nuclear localization drive splicing defects stimulate formation non-amyloid inclusions affected neurons. However, mechanism by which mutations contribute to development ALS remains uncertain. Here we describe a pattern changes dynamics continuous proteinopathy induced mislocalized FUS. We show that...
Constitutive expression of Hsp27 has been demonstrated in vertebrate embryos, especially developing skeletal and cardiac muscle. Results several previous studies have indicated that could play a role the development these tissues. For example, inhibition reported to cause defective mammalian myoblasts vitro frog embryos vivo. In contrast, transgenic mice lacking develop normally. Here, we examined distribution protein adult zebrafish effects suppressing using phosphorodiamidate morpholino...
Mutations to the RNA binding protein, fused in sarcoma (FUS) occur ∼5% of familial ALS and FUS-positive cytoplasmic inclusions are commonly observed these patients. Altered metabolism is increasingly implicated ALS, yet it not understood how specificity with which FUS interacts cytoplasm can affect its aggregation vivo. To further understand this, we expressed, mice, a form (FUS ΔRRMcyt) that lacked recognition motif (RRM), thought impart FUS-RNA interactions, carried an ALS-associated point...
Currently, there are no effective drugs for the treatment of amyotrophic lateral sclerosis (ALS). Only two drugs-edaravone and riluzole-have been approved, but they have very limited efficacy. The aim this work was to modify structural core Edaravone-phenylpyrazolone moiety combine it with aminoadamantane pharmacophore in order expand spectrum its action a number processes involved pathogenesis ALS. New conjugates edaravone derivatives 1-aminoadamantanes combined alkylene or hydroxypropylene...
<i>Background:</i> Recent clinical studies have demonstrated that dimebon, a drug originally designed and used as non-selective antihistamine, ameliorates symptoms delays progress of mild to moderate forms Alzheimer’s Huntington’s diseases. Although the mechanism dimebon action on pathological processes in degenerating brain is elusive, results carried out cell cultures animal models suggested this might affect process accumulation aggregation various proteins involved...