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  1. AU="Clare Duncan"
  2. AU="Calvo Soto, Andrea Patricia"
  3. AU="Joanna I. Olszewska"
  4. AU="Francesco Cavallieri"
  5. AU="Betaieb, Ehssen"
  6. AU="Fan, Xiaoyu"
  7. AU="Riveros-Magaña, Alma Rocío"
  8. AU="Zhang, Wei-Fen"
  9. AU="Ciuca, Catrinel"
  10. AU="Friend, James R"
  11. AU="Colin R. Jackson"
  12. AU="Messina, Claudia"
  13. AU="Faircloth, Chelsey"
  14. AU="Md. Zabirul Islam" AU="Md. Zabirul Islam"
  15. AU="Butcher, Xochitl"
  16. AU="Espay, Alberto J."

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  1. Artikel ; Online: Potential for Return on Investment in Rehabilitation-Oriented Blue Carbon Projects

    Clare Duncan / Jurgenne H. Primavera / Nicholas A. O. Hill / Dominic C. J. Wodehouse / Heather J. Koldewey

    Frontiers in Forests and Global Change, Vol

    Accounting Methodologies and Project Strategies

    2022  Band 4

    Abstract: Opportunities to boost climate change mitigation and adaptation (CCMA) and sustainable conservation financing may lie in enhancing blue carbon sequestration, particularly in developing nations where coastal ecosystems are extensive and international ... ...

    Abstract Opportunities to boost climate change mitigation and adaptation (CCMA) and sustainable conservation financing may lie in enhancing blue carbon sequestration, particularly in developing nations where coastal ecosystems are extensive and international carbon markets offer comparatively attractive payments for environmental stewardship. While blue carbon is receiving increased global attention, few credit-generating projects are operational, due to low credit-buyer incentives with uncertainty in creditable emissions reductions and high project costs. Little empirical guidance exists for practitioners to quantify return-on-investment (ROI) and viability of potential projects, particularly for rehabilitation where multiple implementation options exist with diverse associated costs. We map and model drivers of mangrove natural regeneration (NR) using remote sensing (high-resolution satellite imagery segmentation and time-series modeling), and subsequent carbon sequestration using field- and literature-derived data, across abandoned aquaculture ponds in the Philippines. Using project-specific cost data, we then assess ROI for a hypothetical rehabilitation-focused mangrove blue carbon project at a 9.68 ha abandoned pond over a 10-year timeframe, under varied rehabilitation scenarios [NR vs. assisted natural regeneration (ANR) with planting], potential emissions reduction accreditation methodologies, carbon prices and discount rates. NR was faster in lower-lying ponds with lower tidal exposure (greater pond dike retention). Forecasted carbon sequestration was 3.7- to 5.2-fold and areal “greenbelt” regeneration 2.5- to 3.4-fold greater in our case study under ANR than NR. Variability in modeled sequestration rates drove high uncertainty and credit deductions in NR strategies. ROI with biomass-only accreditation was low and negative under NR and ANR, respectively. ROI was greater under ANR with inclusion of biomass and autochthonous soil carbon; however, neither strategy was highly profitable at current voluntary market ...
    Schlagwörter mangroves ; carbon emissions reduction ; rehabilitation ; natural regeneration ; blue carbon ; remote sensing ; Forestry ; SD1-669.5 ; Environmental sciences ; GE1-350
    Thema/Rubrik (Code) 333
    Sprache Englisch
    Erscheinungsdatum 2022-01-01T00:00:00Z
    Verlag Frontiers Media S.A.
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  2. Artikel ; Online: SmallSats

    David J. Curnick / Alasdair J. Davies / Clare Duncan / Robin Freeman / David M. P. Jacoby / Hugo T. E. Shelley / Cristian Rossi / Oliver R. Wearn / Michael J. Williamson / Nathalie Pettorelli

    Remote Sensing in Ecology and Conservation, Vol 8, Iss 2, Pp 139-

    a new technological frontier in ecology and conservation?

    2022  Band 150

    Abstract: Abstract We are in the midst of a revolution in satellite technology, with the rapid development and advancement of small satellites (or SmallSats, i.e., satellites <180 kg). Here, we review the opportunities and challenges that such technology might ... ...

    Abstract Abstract We are in the midst of a revolution in satellite technology, with the rapid development and advancement of small satellites (or SmallSats, i.e., satellites <180 kg). Here, we review the opportunities and challenges that such technology might afford in the field of conservation and ecology. SmallSat constellations may yield higher resolutions than those that are currently available to scientists and practitioners, increasing opportunities to improve environmental‐monitoring and animal‐tracking capabilities. They may cut access costs to end users, by reducing operational costs and bringing increased competition to the existing market. Their greater flexibility and affordability may moreover enable the development of bespoke constellations for specific conservation and ecological applications, and provide greater interoperability with ground‐based sensors, such as tracking devices and camera traps. In addition, SmallSats may serve as cost‐effective research and development platforms for new components and products. Combined, these benefits could significantly improve our ability to monitor threats to the environment as they unfold, while enhancing our understanding of animal ecology and ecosystem dynamics. However, significant hardware and software developments are required before such technology is able to produce, process and handle reliable and cost‐effective data, and the initial research and development costs still represent a major challenge. Further, we argue that much remains to be done to ensure these new data products become accessible, equitable and sustainable.
    Schlagwörter Biotelemetry ; earth observation ; nanosatellites ; optical imagery ; remote sensing ; small satellites ; Technology ; T ; Ecology ; QH540-549.5
    Thema/Rubrik (Code) 333
    Sprache Englisch
    Erscheinungsdatum 2022-04-01T00:00:00Z
    Verlag Wiley
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  3. Artikel: A New Framework to Assess Relative Ecosystem Vulnerability to Climate Change

    Lee, Calvin Ka Fai / Clare Duncan / Harry Jon Foord Owen / Nathalie Pettorelli

    Conservation letters. 2018 Mar., v. 11, no. 2

    2018  

    Abstract: Climate change poses a growing risk to global biodiversity. To prioritize conservation efforts, identification of the species and ecosystems most at risk from further changes in climatic conditions is critically needed. Although frameworks are available ... ...

    Abstract Climate change poses a growing risk to global biodiversity. To prioritize conservation efforts, identification of the species and ecosystems most at risk from further changes in climatic conditions is critically needed. Although frameworks are available to assess species vulnerability to climate change, we still lack an easily implementable, ecosystem‐level perspective to inform landscape management. Here, we introduce a novel, spatially explicit vulnerability framework able to generate assessments at the ecosystem scale and apply it to Mozambican forest mangroves, which are under growing pressures from climate change. Results show that most of these ecosystems are currently highly vulnerable to sea level rise, while mangroves in the Zambezia and Nampula districts are highly vulnerable to both sea level rise and tropical storms. Altogether, we believe the introduced assessment framework has clear potential to inform conservation planning and management at various spatial scales, and help achieve adaptive management in the face of climatic uncertainties.
    Schlagwörter adaptive management ; biodiversity ; climate change ; climatic factors ; ecosystems ; landscape management ; mangroves ; planning ; risk ; sea level ; storms ; uncertainty
    Sprache Englisch
    Erscheinungsverlauf 2018-03
    Umfang p. e12372.
    Erscheinungsort John Wiley & Sons, Ltd
    Dokumenttyp Artikel
    Anmerkung LETTER
    ISSN 1755-263X
    DOI 10.1111/conl.12372
    Datenquelle NAL Katalog (AGRICOLA)

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  4. Artikel ; Online: Mapping the Extent of Mangrove Ecosystem Degradation by Integrating an Ecological Conceptual Model with Satellite Data

    Calvin K. F. Lee / Clare Duncan / Emily Nicholson / Temilola E. Fatoyinbo / David Lagomasino / Nathan Thomas / Thomas A. Worthington / Nicholas J. Murray

    Remote Sensing, Vol 13, Iss 2047, p

    2021  Band 2047

    Abstract: Anthropogenic and natural disturbances can cause degradation of ecosystems, reducing their capacity to sustain biodiversity and provide ecosystem services. Understanding the extent of ecosystem degradation is critical for estimating risks to ecosystems, ... ...

    Abstract Anthropogenic and natural disturbances can cause degradation of ecosystems, reducing their capacity to sustain biodiversity and provide ecosystem services. Understanding the extent of ecosystem degradation is critical for estimating risks to ecosystems, yet there are few existing methods to map degradation at the ecosystem scale and none using freely available satellite data for mangrove ecosystems. In this study, we developed a quantitative classification model of mangrove ecosystem degradation using freely available earth observation data. Crucially, a conceptual model of mangrove ecosystem degradation was established to identify suitable remote sensing variables that support the quantitative classification model, bridging the gap between satellite-derived variables and ecosystem degradation with explicit ecological links. We applied our degradation model to two case-studies, the mangroves of Rakhine State, Myanmar, which are severely threatened by anthropogenic disturbances, and Shark River within the Everglades National Park, USA, which is periodically disturbed by severe tropical storms. Our model suggested that 40% (597 km 2 ) of the extent of mangroves in Rakhine showed evidence of degradation. In the Everglades, the model suggested that the extent of degraded mangrove forest increased from 5.1% to 97.4% following the Category 4 Hurricane Irma in 2017. Quantitative accuracy assessments indicated the model achieved overall accuracies of 77.6% and 79.1% for the Rakhine and the Everglades, respectively. We highlight that using an ecological conceptual model as the basis for building quantitative classification models to estimate the extent of ecosystem degradation ensures the ecological relevance of the classification models. Our developed method enables researchers to move beyond only mapping ecosystem distribution to condition and degradation as well. These results can help support ecosystem risk assessments, natural capital accounting, and restoration planning and provide quantitative estimates of ...
    Schlagwörter mangrove ; ecosystem assessment ; Myanmar ; Everglades ; satellite imagery ; degradation ; Science ; Q
    Thema/Rubrik (Code) 551
    Sprache Englisch
    Erscheinungsdatum 2021-05-01T00:00:00Z
    Verlag MDPI AG
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  5. Artikel ; Online: Baseline characteristics of people experiencing homelessness with a recent drug overdose in the PHOENIx pilot randomised controlled trial

    Richard Lowrie / Andrew McPherson / Frances S. Mair / Kate Stock / Caitlin Jones / Donogh Maguire / Vibhu Paudyal / Clare Duncan / Becky Blair / Cian Lombard / Steven Ross / Fiona Hughes / Jane Moir / Ailsa Scott / Frank Reilly / Laura Sills / Jennifer Hislop / Natalia Farmer / Sharon Lucey /
    Stephen Wishart / George Provan / Roy Robertson / Andrea Williamson

    Harm Reduction Journal, Vol 20, Iss 1, Pp 1-

    2023  Band 18

    Abstract: Abstract Background Drug-related deaths in Scotland are the highest in Europe. Half of all deaths in people experiencing homelessness are drug related, yet we know little about the unmet health needs of people experiencing homelessness with recent non- ... ...

    Abstract Abstract Background Drug-related deaths in Scotland are the highest in Europe. Half of all deaths in people experiencing homelessness are drug related, yet we know little about the unmet health needs of people experiencing homelessness with recent non-fatal overdose, limiting a tailored practice and policy response to a public health crisis. Methods People experiencing homelessness with at least one non-fatal street drug overdose in the previous 6 months were recruited from 20 venues in Glasgow, Scotland, and randomised into PHOENIx plus usual care, or usual care. PHOENIx is a collaborative assertive outreach intervention by independent prescriber NHS Pharmacists and third sector homelessness workers, offering repeated integrated, holistic physical, mental and addictions health and social care support including prescribing. We describe comprehensive baseline characteristics of randomised participants. Results One hundred and twenty-eight participants had a mean age of 42 years (SD 8.4); 71% male, homelessness for a median of 24 years (IQR 12–30). One hundred and eighteen (92%) lived in large, congregate city centre temporary accommodation. A quarter (25%) were not registered with a General Practitioner. Participants had overdosed a mean of 3.2 (SD 3.2) times in the preceding 6 months, using a median of 3 (IQR 2–4) non-prescription drugs concurrently: 112 (87.5%) street valium (benzodiazepine-type new psychoactive substances); 77 (60%) heroin; and 76 (59%) cocaine. Half (50%) were injecting, 50% into their groins. 90% were receiving care from Alcohol and Drug Recovery Services (ADRS), and in addition to using street drugs, 90% received opioid substitution therapy (OST), 10% diazepam for street valium use and one participant received heroin-assisted treatment. Participants had a mean of 2.2 (SD 1.3) mental health problems and 5.4 (SD 2.5) physical health problems; 50% received treatment for physical or mental health problems. Ninety-one per cent had at least one mental health problem; 66% had no specialist mental ...
    Schlagwörter Homelessness ; Chronic disease ; Opioid addiction ; Primary health care ; Randomised controlled trial ; Public health ; Public aspects of medicine ; RA1-1270
    Thema/Rubrik (Code) 360
    Sprache Englisch
    Erscheinungsdatum 2023-04-01T00:00:00Z
    Verlag BMC
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  6. Artikel ; Online: Predicting the future impact of droughts on ungulate populations in arid and semi-arid environments.

    Clare Duncan / Aliénor L M Chauvenet / Louise M McRae / Nathalie Pettorelli

    PLoS ONE, Vol 7, Iss 12, p e

    2012  Band 51490

    Abstract: Droughts can have a severe impact on the dynamics of animal populations, particularly in semi-arid and arid environments where herbivore populations are strongly limited by resource availability. Increased drought intensity under projected climate change ...

    Abstract Droughts can have a severe impact on the dynamics of animal populations, particularly in semi-arid and arid environments where herbivore populations are strongly limited by resource availability. Increased drought intensity under projected climate change scenarios can be expected to reduce the viability of such populations, yet this impact has seldom been quantified. In this study, we aim to fill this gap and assess how the predicted worsening of droughts over the 21(st) century is likely to impact the population dynamics of twelve ungulate species occurring in arid and semi-arid habitats. Our results provide support to the hypotheses that more sedentary, grazing and mixed feeding species will be put at high risk from future increases in drought intensity, suggesting that management intervention under these conditions should be targeted towards species possessing these traits. Predictive population models for all sedentary, grazing or mixed feeding species in our study show that their probability of extinction dramatically increases under future emissions scenarios, and that this extinction risk is greater for smaller populations than larger ones. Our study highlights the importance of quantifying the current and future impacts of increasing extreme natural events on populations and species in order to improve our ability to mitigate predicted biodiversity loss under climate change.
    Schlagwörter Medicine ; R ; Science ; Q
    Sprache Englisch
    Erscheinungsdatum 2012-01-01T00:00:00Z
    Verlag Public Library of Science (PLoS)
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  7. Artikel ; Online: Neuroprotective copper bis(thiosemicarbazonato) complexes promote neurite elongation.

    Laura Bica / Jeffrey R Liddell / Paul S Donnelly / Clare Duncan / Aphrodite Caragounis / Irene Volitakis / Brett M Paterson / Roberto Cappai / Alexandra Grubman / James Camakaris / Peter J Crouch / Anthony R White

    PLoS ONE, Vol 9, Iss 2, p e

    2014  Band 90070

    Abstract: Abnormal biometal homeostasis is a central feature of many neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD), and motor neuron disease. Recent studies have shown that metal complexing compounds behaving as ... ...

    Abstract Abnormal biometal homeostasis is a central feature of many neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD), and motor neuron disease. Recent studies have shown that metal complexing compounds behaving as ionophores such as clioquinol and PBT2 have robust therapeutic activity in animal models of neurodegenerative disease; however, the mechanism of neuroprotective action remains unclear. These neuroprotective or neurogenerative processes may be related to the delivery or redistribution of biometals, such as copper and zinc, by metal ionophores. To investigate this further, we examined the effect of the bis(thiosemicarbazonato)-copper complex, Cu(II)(gtsm) on neuritogenesis and neurite elongation (neurogenerative outcomes) in PC12 neuronal-related cultures. We found that Cu(II)(gtsm) induced robust neurite elongation in PC12 cells when delivered at concentrations of 25 or 50 nM overnight. Analogous effects were observed with an alternative copper bis(thiosemicarbazonato) complex, Cu(II)(atsm), but at a higher concentration. Induction of neurite elongation by Cu(II)(gtsm) was restricted to neurites within the length range of 75-99 µm with a 2.3-fold increase in numbers of neurites in this length range with 50 nM Cu(II)(gtsm) treatment. The mechanism of neurogenerative action was investigated and revealed that Cu(II)(gtsm) inhibited cellular phosphatase activity. Treatment of cultures with 5 nM FK506 (calcineurin phosphatase inhibitor) resulted in analogous elongation of neurites compared to 50 nM Cu(II)(gtsm), suggesting a potential link between Cu(II)(gtsm)-mediated phosphatase inhibition and neurogenerative outcomes.
    Schlagwörter Medicine ; R ; Science ; Q
    Sprache Englisch
    Erscheinungsdatum 2014-01-01T00:00:00Z
    Verlag Public Library of Science (PLoS)
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  8. Artikel ; Online: Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis

    Katja M. Kanninen / Alexandra Grubman / Aphrodite Caragounis / Clare Duncan / Sarah J. Parker / Grace E. Lidgerwood / Irene Volitakis / George Ganio / Peter J. Crouch / Anthony R. White

    Biology Open, Vol 2, Iss 6, Pp 635-

    2013  Band 646

    Abstract: Summary Neuronal ceroid lipofuscinoses, the most common fatal childhood neurodegenerative illnesses, share many features with more prevalent neurodegenerative diseases. Neuronal ceroid lipofuscinoses are caused by mutations in CLN genes. CLN6 encodes a ... ...

    Abstract Summary Neuronal ceroid lipofuscinoses, the most common fatal childhood neurodegenerative illnesses, share many features with more prevalent neurodegenerative diseases. Neuronal ceroid lipofuscinoses are caused by mutations in CLN genes. CLN6 encodes a transmembrane endoplasmic reticulum protein with no known function. We characterized the behavioural phenotype of spontaneous mutant mice modeling CLN6 disease, and demonstrate progressive motor and visual decline and reduced lifespan in these mice, consistent with symptoms observed in neuronal ceroid lipofuscinosis patients. Alterations to biometal homeostasis are known to play a critical role in pathology in Alzheimer's, Parkinson's, Huntington's and motor neuron diseases. We have previously shown accumulation of the biometals, zinc, copper, manganese and cobalt, in CLN6 Merino and South Hampshire sheep at the age of symptom onset. Here we determine the physiological and disease-associated expression of CLN6, demonstrating regional CLN6 transcript loss, and concurrent accumulation of the same biometals in the CNS and the heart of presymptomatic CLN6 mice. Furthermore, increased expression of the ER/Golgi-localized cation transporter protein, Zip7, was detected in cerebellar Purkinje cells and whole brain fractions. Purkinje cells not only control motor function, an early symptomatic change in the CLN6 mice, but also display prominent neuropathological changes in mouse models and patients with different forms of neuronal ceroid lipofuscinoses. Whole brain fractionation analysis revealed biometal accumulation in fractions expressing markers for ER, Golgi, endosomes and lysosomes of CLN6 brains. These data are consistent with a link between CLN6 expression and biometal homeostasis in CLN6 disease, and provide further support for altered cation transporter regulation as a key factor in neurodegeneration.
    Schlagwörter Neurodegeneration ; Biometal homeostasis ; Neuronal ceroid lipofuscinoses ; CLN6 ; Metal transporter ; Science ; Q ; Biology (General) ; QH301-705.5
    Thema/Rubrik (Code) 616
    Sprache Englisch
    Erscheinungsdatum 2013-05-01T00:00:00Z
    Verlag The Company of Biologists
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  9. Artikel ; Online: Kinase Inhibitor Screening Identifies Cyclin-Dependent Kinases and Glycogen Synthase Kinase 3 as Potential Modulators of TDP-43 Cytosolic Accumulation during Cell Stress.

    Diane Moujalled / Janine L James / Sarah J Parker / Grace E Lidgerwood / Clare Duncan / Jodi Meyerowitz / Takashi Nonaka / Masato Hasegawa / Katja M Kanninen / Alexandra Grubman / Jeffrey R Liddell / Peter J Crouch / Anthony R White

    PLoS ONE, Vol 8, Iss 6, p e

    2013  Band 67433

    Abstract: Abnormal processing of TAR DNA binding protein 43 (TDP-43) has been identified as a major factor in neuronal degeneration during amyotrophic lateral sclerosis (ALS) or frontotemporal lobar degeneration (FTLD). It is unclear how changes to TDP-43, ... ...

    Abstract Abnormal processing of TAR DNA binding protein 43 (TDP-43) has been identified as a major factor in neuronal degeneration during amyotrophic lateral sclerosis (ALS) or frontotemporal lobar degeneration (FTLD). It is unclear how changes to TDP-43, including nuclear to cytosolic translocation and subsequent accumulation, are controlled in these diseases. TDP-43 is a member of the heterogeneous ribonucleoprotein (hnRNP) RNA binding protein family and is known to associate with cytosolic RNA stress granule proteins in ALS and FTLD. hnRNP trafficking and accumulation is controlled by the action of specific kinases including members of the mitogen-activated protein kinase (MAPK) pathway. However, little is known about how kinase pathways control TDP-43 movement and accumulation. In this study, we used an in vitro model of TDP-43-positve stress granule formation to screen for the effect of kinase inhibitors on TDP-43 accumulation. We found that while a number of kinase inhibitors, particularly of the MAPK pathways modulated both TDP-43 and the global stress granule marker, human antigen R (HuR), multiple inhibitors were more specific to TDP-43 accumulation, including inhibitors of cyclin-dependent kinases (CDKs) and glycogen synthase kinase 3 (GSK3). Close correlation was observed between effects of these inhibitors on TDP-43, hnRNP K and TIAR, but often with different effects on HuR accumulation. This may indicate a potential interaction between TDP-43, hnRNP K and TIAR. CDK inhibitors were also found to reverse pre-formed TDP-43-positive stress granules and both CDK and GSK3 inhibitors abrogated the accumulation of C-terminal TDP-43 (219-414) in transfected cells. Further studies are required to confirm the specific kinases involved and whether their action is through phosphorylation of the TDP-43 binding partner hnRNP K. This knowledge provides a valuable insight into the mechanisms controlling abnormal cytoplasmic TDP-43 accumulation and may herald new opportunities for kinase modulation-based therapeutic ...
    Schlagwörter Medicine ; R ; Science ; Q
    Thema/Rubrik (Code) 571
    Sprache Englisch
    Erscheinungsdatum 2013-01-01T00:00:00Z
    Verlag Public Library of Science (PLoS)
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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  10. Artikel ; Online: Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis.

    Katja M Kanninen / Alexandra Grubman / Jodi Meyerowitz / Clare Duncan / Jiang-Li Tan / Sarah J Parker / Peter J Crouch / Brett M Paterson / James L Hickey / Paul S Donnelly / Irene Volitakis / Imke Tammen / David N Palmer / Anthony R White

    PLoS ONE, Vol 8, Iss 3, p e

    2013  Band 58644

    Abstract: Mutations in the CLN6 gene cause a variant late infantile form of neuronal ceroid lipofuscinosis (NCL; Batten disease). CLN6 loss leads to disease clinically characterized by vision impairment, motor and cognitive dysfunction, and seizures. Accumulating ... ...

    Abstract Mutations in the CLN6 gene cause a variant late infantile form of neuronal ceroid lipofuscinosis (NCL; Batten disease). CLN6 loss leads to disease clinically characterized by vision impairment, motor and cognitive dysfunction, and seizures. Accumulating evidence suggests that alterations in metal homeostasis and cellular signaling pathways are implicated in several neurodegenerative and developmental disorders, yet little is known about their role in the NCLs. To explore the disease mechanisms of CLN6 NCL, metal concentrations and expression of proteins implicated in cellular signaling pathways were assessed in brain tissue from South Hampshire and Merino CLN6 sheep. Analyses revealed increased zinc and manganese concentrations in affected sheep brain in those regions where neuroinflammation and neurodegeneration first occur. Synaptic proteins, the metal-binding protein metallothionein, and the Akt/GSK3 and ERK/MAPK cellular signaling pathways were also altered. These results demonstrate that altered metal concentrations, synaptic protein changes, and aberrant modulation of cellular signaling pathways are characteristic features in the CLN6 ovine form of NCL.
    Schlagwörter Medicine ; R ; Science ; Q
    Thema/Rubrik (Code) 572
    Sprache Englisch
    Erscheinungsdatum 2013-01-01T00:00:00Z
    Verlag Public Library of Science (PLoS)
    Dokumenttyp Artikel ; Online
    Datenquelle BASE - Bielefeld Academic Search Engine (Lebenswissenschaftliche Auswahl)

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