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  1. Article ; Online: Economic evaluation of Motor Neuron Diseases: a nationwide cross-sectional analysis in Germany.

    Heinrich, Felix / Cordts, Isabell / Günther, René / Stolte, Benjamin / Zeller, Daniel / Schröter, Carsten / Weyen, Ute / Regensburger, Martin / Wolf, Joachim / Schneider, Ilka / Hermann, Andreas / Metelmann, Moritz / Kohl, Zacharias / Linker, Ralf A / Koch, Jan Christoph / Radelfahr, Florentine / Schönfelder, Erik / Gardt, Pavel / Mohajer-Peseschkian, Tara /
    Osmanovic, Alma / Klopstock, Thomas / Dorst, Johannes / Ludolph, Albert C / Schöffski, Oliver / Boentert, Matthias / Hagenacker, Tim / Deschauer, Marcus / Lingor, Paul / Petri, Susanne / Schreiber-Katz, Olivia

    Journal of neurology

    2023  Volume 270, Issue 10, Page(s) 4922–4938

    Abstract: Background and objectives: Motor Neuron Diseases (MND) are rare diseases but have a high impact on affected individuals and society. This study aims to perform an economic evaluation of MND in Germany.: Methods: Primary patient-reported data were ... ...

    Abstract Background and objectives: Motor Neuron Diseases (MND) are rare diseases but have a high impact on affected individuals and society. This study aims to perform an economic evaluation of MND in Germany.
    Methods: Primary patient-reported data were collected including individual impairment, the use of medical and non-medical resources, and self-rated Health-Related Quality of Life (HRQoL). Annual socio-economic costs per year as well as Quality-Adjusted Life Years (QALYs) were calculated.
    Results: 404 patients with a diagnosis of Amyotrophic Lateral Sclerosis (ALS), Spinal Muscular Atrophy (SMA) or Hereditary Spastic Paraplegia (HSP) were enrolled. Total annual costs per patient were estimated at 83,060€ in ALS, 206,856€ in SMA and 27,074€ in HSP. The main cost drivers were informal care (all MND) and disease-modifying treatments (SMA). Self-reported HRQoL was best in patients with HSP (mean EuroQoL Five Dimension Five Level (EQ-5D-5L) index value 0.67) and lowest in SMA patients (mean EQ-5D-5L index value 0.39). QALYs for patients with ALS were estimated to be 1.89 QALYs, 23.08 for patients with HSP and 14.97 for patients with SMA, respectively. Cost-utilities were estimated as follows: 138,960€/QALY for ALS, 525,033€/QALY for SMA, and 49,573€/QALY for HSP. The main predictors of the high cost of illness and low HRQoL were disease progression and loss of individual autonomy.
    Conclusion: As loss of individual autonomy was the main cost predictor, therapeutic and supportive measures to maintain this autonomy may contribute to reducing high personal burden and also long-term costs, e.g., care dependency and absenteeism from work.
    MeSH term(s) Humans ; Quality of Life ; Cost of Illness ; Cross-Sectional Studies ; Amyotrophic Lateral Sclerosis ; Cost-Benefit Analysis ; Surveys and Questionnaires ; Health Care Costs ; Germany/epidemiology ; Muscular Atrophy, Spinal
    Language English
    Publishing date 2023-06-25
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 187050-6
    ISSN 1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
    ISSN (online) 1432-1459
    ISSN 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
    DOI 10.1007/s00415-023-11811-1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Site-directed genotype screening for elimination of antinutritional saponins in quinoa seeds identifies TSARL1 as a master controller of saponin biosynthesis selectively in seeds.

    Trinh, Mai Duy Luu / Visintainer, Davide / Günther, Jan / Østerberg, Jeppe Thulin / da Fonseca, Rute R / Fondevilla, Sara / Moog, Max William / Luo, Guangbin / Nørrevang, Anton F / Crocoll, Christoph / Nielsen, Philip V / Jacobsen, Sven-Erik / Wendt, Toni / Bak, Søren / López-Marqués, Rosa Laura / Palmgren, Michael

    Plant biotechnology journal

    2024  

    Abstract: Climate change may result in a drier climate and increased salinization, threatening agricultural productivity worldwide. Quinoa (Chenopodium quinoa) produces highly nutritious seeds and tolerates abiotic stresses such as drought and high salinity, ... ...

    Abstract Climate change may result in a drier climate and increased salinization, threatening agricultural productivity worldwide. Quinoa (Chenopodium quinoa) produces highly nutritious seeds and tolerates abiotic stresses such as drought and high salinity, making it a promising future food source. However, the presence of antinutritional saponins in their seeds is an undesirable trait. We mapped genes controlling seed saponin content to a genomic region that includes TSARL1. We isolated desired genetic variation in this gene by producing a large mutant library of a commercial quinoa cultivar and screening the library for specific nucleotide substitutions using droplet digital PCR. We were able to rapidly isolate two independent tsarl1 mutants, which retained saponins in the leaves and roots for defence, but saponins were undetectable in the seed coat. We further could show that TSARL1 specifically controls seed saponin biosynthesis in the committed step after 2,3-oxidosqualene. Our work provides new important knowledge on the function of TSARL1 and represents a breakthrough for quinoa breeding.
    Language English
    Publishing date 2024-04-04
    Publishing country England
    Document type Journal Article
    ZDB-ID 2136367-5
    ISSN 1467-7652 ; 1467-7652
    ISSN (online) 1467-7652
    ISSN 1467-7652
    DOI 10.1111/pbi.14340
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Beta-amyloid oligomers and cellular prion protein in Alzheimer's disease.

    Gunther, Erik C / Strittmatter, Stephen M

    Journal of molecular medicine (Berlin, Germany)

    2009  Volume 88, Issue 4, Page(s) 331–338

    Abstract: ... prion protein (PrP(C)) has been shown to act as a functional receptor for A beta oligomers in brain slices ... Because PrP(C) serves as the substrate for Creutzfeldt-Jakob Disease (CJD), these data suggest mechanistic ... in AD, commonalities between AD and CJD, and the newly emergent role of PrP(C) as a receptor for A beta ...

    Abstract Prefibrillar oligomers of the beta-amyloid peptide (A beta) are recognized as potential mediators of Alzheimer's disease (AD) pathophysiology. Deficits in synaptic function, neurotoxicity, and the progression of AD have all been linked to the oligomeric A beta assemblies rather than to A beta monomers or to amyloid plaques. However, the molecular sites of A beta oligomer action have remained largely unknown. Recently, the cellular prion protein (PrP(C)) has been shown to act as a functional receptor for A beta oligomers in brain slices. Because PrP(C) serves as the substrate for Creutzfeldt-Jakob Disease (CJD), these data suggest mechanistic similarities between the two neurodegenerative diseases. Here, we review the importance of A beta oligomers in AD, commonalities between AD and CJD, and the newly emergent role of PrP(C) as a receptor for A beta oligomers.
    MeSH term(s) Alzheimer Disease/blood ; Alzheimer Disease/metabolism ; Amyloid beta-Peptides/chemistry ; Amyloid beta-Peptides/genetics ; Animals ; Brain/pathology ; COS Cells ; Chlorocebus aethiops ; Creutzfeldt-Jakob Syndrome/metabolism ; Humans ; Mice ; Mice, Transgenic ; Neurodegenerative Diseases/pathology ; Peptide Fragments/chemistry ; PrPC Proteins/chemistry ; Prions/chemistry ; Prions/metabolism
    Chemical Substances Amyloid beta-Peptides ; Peptide Fragments ; PrPC Proteins ; Prions ; amyloid beta-protein (1-42)
    Language English
    Publishing date 2009-12-04
    Publishing country Germany
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1223802-8
    ISSN 1432-1440 ; 0946-2716
    ISSN (online) 1432-1440
    ISSN 0946-2716
    DOI 10.1007/s00109-009-0568-7
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Systemic mesalazine treatment prevents spontaneous skin fibrosis in PLK2-deficient mice.

    Newe, Manja / Kant, Theresa A / Hoffmann, Maximilian / Rausch, Johanna S E / Winter, Luise / Künzel, Karolina / Klapproth, Erik / Günther, Claudia / Künzel, Stephan R

    Naunyn-Schmiedeberg's archives of pharmacology

    2021  Volume 394, Issue 11, Page(s) 2233–2244

    Abstract: Skin fibrosis is a complex biological remodeling process occurring in disease like systemic sclerosis, morphea, or eosinophilic fasciitis. Since the knowledge about the underlying pathomechanisms is still incomplete, there is currently no therapy, which ... ...

    Abstract Skin fibrosis is a complex biological remodeling process occurring in disease like systemic sclerosis, morphea, or eosinophilic fasciitis. Since the knowledge about the underlying pathomechanisms is still incomplete, there is currently no therapy, which prevents or reverses skin fibrosis sufficiently. The present study investigates the role of polo-like kinase 2 (PLK2) and the pro-fibrotic cytokine osteopontin (OPN) in the pathogenesis of cutaneous fibrosis and demonstrates the antifibrotic effects of systemic mesalazine treatment in vivo. Isolated primary dermal fibroblasts of PLK2 wild-type (WT) and knockout (KO) mice were characterized in vitro. Skin thickness and histoarchitecture were studied in paraffin-embedded skin sections. The effects of mesalazine treatment were examined in isolated fibroblasts and PLK2 KO mice, which were fed 100 µg/g mesalazine for 6 months via the drinking water. Compared to WT, PLK2 KO fibroblasts displayed higher spontaneous myofibroblast differentiation, reduced proliferation rates, and overexpression of the fibrotic cytokine OPN. In vitro, 72 h of treatment with 10 mmol/L mesalazine induced phenotype conversion in PLK2 KO fibroblasts and attenuated OPN expression by inhibiting ERK1/2. In vivo, dermal myofibroblast differentiation, collagen accumulation, and skin thickening were prevented by mesalazine in PLK2 KO. Plasma creatinine levels indicated good tolerability of systemic long-term mesalazine treatment. The current study reveals a spontaneous fibrotic skin phenotype and ERK1/2-dependent OPN overexpression in PLK2 KO mice. We provide experimental evidence for the antifibrotic effectiveness of systemic mesalazine treatment to prevent fibrosis of the skin, suggesting further investigation in experimental and clinical settings.
    MeSH term(s) Animals ; Anti-Inflammatory Agents, Non-Steroidal/administration & dosage ; Anti-Inflammatory Agents, Non-Steroidal/pharmacology ; Anti-Inflammatory Agents, Non-Steroidal/toxicity ; Cell Differentiation/drug effects ; Collagen/metabolism ; Creatinine/blood ; Disease Models, Animal ; Female ; Fibroblasts/drug effects ; Fibroblasts/pathology ; Fibrosis/prevention & control ; Male ; Mesalamine/administration & dosage ; Mesalamine/pharmacology ; Mesalamine/toxicity ; Mice ; Mice, Knockout ; Osteopontin/genetics ; Protein Serine-Threonine Kinases/genetics ; Skin/drug effects ; Skin/pathology
    Chemical Substances Anti-Inflammatory Agents, Non-Steroidal ; Osteopontin (106441-73-0) ; Mesalamine (4Q81I59GXC) ; Collagen (9007-34-5) ; Creatinine (AYI8EX34EU) ; Protein Serine-Threonine Kinases (EC 2.7.11.1) ; serum-inducible kinase (EC 2.7.11.21)
    Language English
    Publishing date 2021-08-19
    Publishing country Germany
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 121471-8
    ISSN 1432-1912 ; 0028-1298
    ISSN (online) 1432-1912
    ISSN 0028-1298
    DOI 10.1007/s00210-021-02135-w
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Sensitivity of Ultrasonic Coda Wave Interferometry to Material Damage-Observations from a Virtual Concrete Lab.

    Finger, Claudia / Saydak, Leslie / Vu, Giao / Timothy, Jithender J / Meschke, Günther / Saenger, Erik H

    Materials (Basel, Switzerland)

    2021  Volume 14, Issue 14

    Abstract: Ultrasonic measurements are used in civil engineering for structural health monitoring of concrete infrastructures. The late portion of the ultrasonic wavefield, the coda, is sensitive to small changes in the elastic moduli of the material. Coda Wave ... ...

    Abstract Ultrasonic measurements are used in civil engineering for structural health monitoring of concrete infrastructures. The late portion of the ultrasonic wavefield, the coda, is sensitive to small changes in the elastic moduli of the material. Coda Wave Interferometry (CWI) correlates these small changes in the coda with the wavefield recorded in intact, or unperturbed, concrete specimen to reveal the amount of velocity change that occurred. CWI has the potential to detect localized damages and global velocity reductions alike. In this study, the sensitivity of CWI to different types of concrete mesostructures and their damage levels is investigated numerically. Realistic numerical concrete models of concrete specimen are generated, and damage evolution is simulated using the discrete element method. In the virtual concrete lab, the simulated ultrasonic wavefield is propagated from one transducer using a realistic source signal and recorded at a second transducer. Different damage scenarios reveal a different slope in the decorrelation of waveforms with the observed reduction in velocities in the material. Finally, the impact and possible generalizations of the findings are discussed, and recommendations are given for a potential application of CWI in concrete at structural scale.
    Language English
    Publishing date 2021-07-19
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2487261-1
    ISSN 1996-1944
    ISSN 1996-1944
    DOI 10.3390/ma14144033
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  6. Article ; Online: Rescue of Transgenic Alzheimer's Pathophysiology by Polymeric Cellular Prion Protein Antagonists.

    Gunther, Erik C / Smith, Levi M / Kostylev, Mikhail A / Cox, Timothy O / Kaufman, Adam C / Lee, Suho / Folta-Stogniew, Ewa / Maynard, George D / Um, Ji Won / Stagi, Massimiliano / Heiss, Jacqueline K / Stoner, Austin / Noble, Geoff P / Takahashi, Hideyuki / Haas, Laura T / Schneekloth, John S / Merkel, Janie / Teran, Christopher / Naderi, Zahra K /
    Supattapone, Surachai / Strittmatter, Stephen M

    Cell reports

    2020  Volume 26, Issue 1, Page(s) 145–158.e8

    Abstract: Cellular prion protein ( ... ...

    Abstract Cellular prion protein (PrP
    MeSH term(s) Alzheimer Disease/physiopathology ; Animals ; Mice ; Mice, Transgenic ; Prion Proteins/antagonists & inhibitors ; Signal Transduction
    Chemical Substances Prion Proteins
    Language English
    Publishing date 2020-01-28
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2649101-1
    ISSN 2211-1247 ; 2211-1247
    ISSN (online) 2211-1247
    ISSN 2211-1247
    DOI 10.1016/j.celrep.2018.12.021
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Distinct gradients of various neurotransmitter markers in caudate nucleus and putamen of the human brain.

    Hörtnagl, Heide / Pifl, Christian / Hörtnagl, Erik / Reiner, Angelika / Sperk, Günther

    Journal of neurochemistry

    2019  Volume 152, Issue 6, Page(s) 650–662

    Abstract: The caudate nucleus (CN) and the putamen (PUT) as parts of the human striatum are distinguished by a marked heterogeneity in functional, anatomical, and neurochemical patterns. Our study aimed to document in detail the regional diversity in the ... ...

    Abstract The caudate nucleus (CN) and the putamen (PUT) as parts of the human striatum are distinguished by a marked heterogeneity in functional, anatomical, and neurochemical patterns. Our study aimed to document in detail the regional diversity in the distribution of dopamine (DA), serotonin, γ-aminobuturic acid, and choline acetyltransferase within the CN and PUT. For this purpose we dissected the CN as well as the PUT of 12 post-mortem brains of human subjects with no evidence of neurological and psychiatric disorders (38-81 years old) into about 80 tissue parts. We then investigated rostro-caudal, dorso-ventral, and medio-lateral gradients of these neurotransmitter markers. All parameters revealed higher levels, turnover rates, or activities in the PUT than in the CN. Within the PUT, DA levels increased continuously from rostral to caudal. In contrast, the lowest molar ratio of homovanillic acid to DA, a marker of DA turnover, coincided with highest DA levels in the caudal PUT, the part of the striatum with the highest loss of DA in Parkinson's disease (N. Engl. J. Med., 318, 1988, 876). Highest DA concentrations were found in the most central areas both in the PUT and CN. We observed an age-dependent loss of DA in the PUT and CN that did not correspond to the loss described for Parkinson's disease indicating different mechanisms inducing the deficit of DA. Our data demonstrate a marked heterogeneity in the anatomical distribution of neurotransmitter markers in the human dorsal striatum indicating anatomical and functional diversity within this brain structure.
    MeSH term(s) Adult ; Aged ; Aged, 80 and over ; Aging/physiology ; Biomarkers/analysis ; Caudate Nucleus/chemistry ; Caudate Nucleus/physiology ; Choline O-Acetyltransferase/analysis ; Dopamine/analysis ; Female ; Humans ; Male ; Middle Aged ; Neurotransmitter Agents/analysis ; Parkinson Disease/metabolism ; Postmortem Changes ; Putamen/chemistry ; Putamen/physiology ; Serotonin/analysis ; gamma-Aminobutyric Acid/analysis
    Chemical Substances Biomarkers ; Neurotransmitter Agents ; Serotonin (333DO1RDJY) ; gamma-Aminobutyric Acid (56-12-2) ; Choline O-Acetyltransferase (EC 2.3.1.6) ; Dopamine (VTD58H1Z2X)
    Language English
    Publishing date 2019-11-28
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80158-6
    ISSN 1471-4159 ; 0022-3042 ; 1474-1644
    ISSN (online) 1471-4159
    ISSN 0022-3042 ; 1474-1644
    DOI 10.1111/jnc.14897
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: MondoA drives malignancy in B-ALL through enhanced adaptation to metabolic stress.

    Sipol, Alexandra / Hameister, Erik / Xue, Busheng / Hofstetter, Julia / Barenboim, Maxim / Öllinger, Rupert / Jain, Gaurav / Prexler, Carolin / Rubio, Rebeca Alba / Baldauf, Michaela C / Franchina, Davide G / Petry, Andreas / Schmäh, Juliane / Thiel, Uwe / Görlach, Agnes / Cario, Gunnar / Brenner, Dirk / Richter, Günther H S / Grünewald, Thomas G P /
    Rad, Roland / Wolf, Elmar / Ruland, Jürgen / Sorensen, Poul H / Burdach, Stefan E G

    Blood

    2021  Volume 139, Issue 8, Page(s) 1184–1197

    Abstract: Cancer cells are in most instances characterized by rapid proliferation and uncontrolled cell division. Hence, they must adapt to proliferation-induced metabolic stress through intrinsic or acquired antimetabolic stress responses to maintain homeostasis ... ...

    Abstract Cancer cells are in most instances characterized by rapid proliferation and uncontrolled cell division. Hence, they must adapt to proliferation-induced metabolic stress through intrinsic or acquired antimetabolic stress responses to maintain homeostasis and survival. One mechanism to achieve this is reprogramming gene expression in a metabolism-dependent manner. MondoA (also known as Myc-associated factor X-like protein X-interacting protein [MLXIP]), a member of the MYC interactome, has been described as an example of such a metabolic sensor. However, the role of MondoA in malignancy is not fully understood and the underlying mechanism in metabolic responses remains elusive. By assessing patient data sets, we found that MondoA overexpression is associated with worse survival in pediatric common acute lymphoblastic leukemia (ALL; B-precursor ALL [B-ALL]). Using clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated protein 9 (Cas9) and RNA-interference approaches, we observed that MondoA depletion reduces the transformational capacity of B-ALL cells in vitro and dramatically inhibits malignant potential in an in vivo mouse model. Interestingly, reduced expression of MondoA in patient data sets correlated with enrichment in metabolic pathways. The loss of MondoA correlated with increased tricarboxylic acid cycle activity. Mechanistically, MondoA senses metabolic stress in B-ALL cells by restricting oxidative phosphorylation through reduced pyruvate dehydrogenase activity. Glutamine starvation conditions greatly enhance this effect and highlight the inability to mitigate metabolic stress upon loss of MondoA in B-ALL. Our findings give novel insight into the function of MondoA in pediatric B-ALL and support the notion that MondoA inhibition in this entity offers a therapeutic opportunity and should be further explored.
    MeSH term(s) Animals ; Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics ; Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/metabolism ; Cell Line, Tumor ; Humans ; Mice ; Mice, Inbred BALB C ; Mice, Knockout ; Neoplasm Proteins/genetics ; Neoplasm Proteins/metabolism ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/genetics ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/metabolism ; Stress, Physiological
    Chemical Substances Basic Helix-Loop-Helix Leucine Zipper Transcription Factors ; MLXIP protein, human ; Neoplasm Proteins
    Language English
    Publishing date 2021-05-19
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2020007932
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  9. Article: A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany.

    Peseschkian, Tara / Cordts, Isabell / Günther, René / Stolte, Benjamin / Zeller, Daniel / Schröter, Carsten / Weyen, Ute / Regensburger, Martin / Wolf, Joachim / Schneider, Ilka / Hermann, Andreas / Metelmann, Moritz / Kohl, Zacharias / Linker, Ralf A / Koch, Jan Christoph / Büchner, Boriana / Weiland, Ulrike / Schönfelder, Erik / Heinrich, Felix /
    Osmanovic, Alma / Klopstock, Thomas / Dorst, Johannes / Ludolph, Albert C / Boentert, Matthias / Hagenacker, Tim / Deschauer, Marcus / Lingor, Paul / Petri, Susanne / Schreiber-Katz, Olivia

    Brain sciences

    2021  Volume 11, Issue 3

    Abstract: Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL ( ... ...

    Abstract Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL (HRQoL) in 325 ALS patients was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5) and EuroQol Five Dimension Five Level Scale (EQ-5D-5L), together with disease severity (captured by the revised ALS Functional Rating Scale (ALSFRS-R)) and the current care and therapies used by our cohort. At inclusion, the mean ALSAQ-5 total score was 56.93 (max. 100, best = 0) with a better QoL associated with a less severe disease status (β = -1.96 per increase of one point in the ALSFRS-R score,
    Language English
    Publishing date 2021-03-14
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2651993-8
    ISSN 2076-3425
    ISSN 2076-3425
    DOI 10.3390/brainsci11030372
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  10. Article ; Online: Liquid and Hydrogel Phases of PrP

    Kostylev, Mikhail A / Tuttle, Marcus D / Lee, Suho / Klein, Lauren E / Takahashi, Hideyuki / Cox, Timothy O / Gunther, Erik C / Zilm, Kurt W / Strittmatter, Stephen M

    Molecular cell

    2018  Volume 72, Issue 3, Page(s) 426–443.e12

    Abstract: Protein phase separation by low-complexity, intrinsically disordered domains generates membraneless organelles and links to neurodegeneration. Cellular prion protein ( ... ...

    Abstract Protein phase separation by low-complexity, intrinsically disordered domains generates membraneless organelles and links to neurodegeneration. Cellular prion protein (PrP
    MeSH term(s) Alzheimer Disease/metabolism ; Amyloid beta-Peptides/physiology ; Animals ; Brain ; COS Cells ; Cell Line ; Cell Membrane ; Chlorocebus aethiops ; HEK293 Cells ; Humans ; Hydrogels ; Magnetic Resonance Imaging/methods ; Molecular Conformation ; Neurons ; PrPC Proteins/chemistry ; PrPC Proteins/physiology ; Prions/chemistry ; Prions/physiology ; Protein Binding ; Receptor, Metabotropic Glutamate 5 ; Signal Transduction
    Chemical Substances Amyloid beta-Peptides ; Hydrogels ; PrPC Proteins ; Prions ; Receptor, Metabotropic Glutamate 5
    Language English
    Publishing date 2018-10-25
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 1415236-8
    ISSN 1097-4164 ; 1097-2765
    ISSN (online) 1097-4164
    ISSN 1097-2765
    DOI 10.1016/j.molcel.2018.10.009
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