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  1. Book: Notch signaling

    Bellen, Hugo J.

    methods and protocols

    (Methods in molecular biology ; 1187)

    2014  

    Author's details ed. by Hugo J. Bellen
    Series title Methods in molecular biology ; 1187
    Collection
    Keywords Notch proteins ; Notch genes ; Cellular signal transduction
    Subject code 571.6
    Language English
    Size XI, 351 S. : Ill., graph. Darst.
    Publisher Humana Press
    Publishing place New York u.a.
    Publishing country United States
    Document type Book
    HBZ-ID HT018341499
    ISBN 978-1-4939-1138-7 ; 9781493911394 ; 1-4939-1138-4 ; 1493911392
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Zs A 7042 -1187- verfügbar in Königswinter Königswinter

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  2. Article ; Online: Lord of the fruit flies: an interview with Hugo Bellen.

    Bellen, Hugo J

    Disease models & mechanisms

    2022  Volume 15, Issue 3

    Abstract: During his remarkable career, Professor Hugo Bellen has innovated Drosophila genetics and forged a community driven toward diagnosis and treatment of rare diseases. He has advanced our understanding of nervous system development and neurodegeneration by ... ...

    Abstract During his remarkable career, Professor Hugo Bellen has innovated Drosophila genetics and forged a community driven toward diagnosis and treatment of rare diseases. He has advanced our understanding of nervous system development and neurodegeneration by exploring mechanisms and genetics through the latticed eyes of the common fruit fly. His lab, along with the labs of Shinya Yamamoto and Michael Wangler at Baylor College of Medicine and the Jan and Dan Duncan Neurological Research Institute of Texas Children's Hospital in Houston, also function as the Drosophila Core of the Model Organisms Screening Center (MOSC) of the Undiagnosed Diseases Network (UDN) and the Center for Precision Medicine Models. In this capacity, they facilitate the diagnosis of (ultra)rare human diseases and contribute to the development of treatments for these patients. Hugo is also the head of the Drosophila Gene Disruption Project supported by the National Institutes of Health (NIH) Office of Research Infrastructure Programs, and his lab channels substantial resources to the development of novel and sophisticated tools and technology that are then shared openly with the community via the Bloomington Drosophila Stock Center and the Drosophila Genomics Resource Center to propel research across the globe. Hugo has received an array of awards for his contributions to science and medicine, and he continues to be one of the most prominent figures in translational model organism research. In this interview, he discusses how his career progressed towards Drosophila genetics and highlights the accomplishments and challenges faced by the model organism community.
    MeSH term(s) Animals ; Drosophila ; Genomics ; Humans ; Precision Medicine ; Rare Diseases/genetics ; Undiagnosed Diseases
    Language English
    Publishing date 2022-03-18
    Publishing country England
    Document type Interview
    ZDB-ID 2451104-3
    ISSN 1754-8411 ; 1754-8403
    ISSN (online) 1754-8411
    ISSN 1754-8403
    DOI 10.1242/dmm.049500
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Glial lipid droplets resolve ROS during sleep.

    Goodman, Lindsey D / Moulton, Matthew J / Bellen, Hugo J

    Nature neuroscience

    2024  Volume 27, Issue 4, Page(s) 610–612

    MeSH term(s) Reactive Oxygen Species/metabolism ; Lipid Droplets/metabolism ; Neuroglia ; Neurons ; Sleep ; Lipid Metabolism
    Chemical Substances Reactive Oxygen Species
    Language English
    Publishing date 2024-02-01
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1420596-8
    ISSN 1546-1726 ; 1097-6256
    ISSN (online) 1546-1726
    ISSN 1097-6256
    DOI 10.1038/s41593-023-01546-7
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4891: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MG 67: Show issues

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  4. Book: Neurotransmitter release

    Bellen, Hugo J.

    (Frontiers in molecular biology ; 23)

    1999  

    Author's details ed. by Hugo J. Bellen
    Series title Frontiers in molecular biology ; 23
    Collection
    Keywords Neurotransmitter ; Synaptische Transmission
    Subject Neurotransmission ; Synaptische Übertragung ; Transmitter
    Language English
    Size XVIII, 437 S. : Ill., graph. Darst.
    Publisher Oxford Univ. Press
    Publishing place Oxford u.a.
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT011139239
    ISBN 0-19-963767-9 ; 0-19-963766-0 ; 978-0-19-963767-6 ; 978-0-19-963766-9
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    1999 A 6924 order for loan Magazin

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  5. Article ; Online: Recent insights into the role of glia and oxidative stress in Alzheimer's disease gained from Drosophila.

    Goodman, Lindsey D / Bellen, Hugo J

    Current opinion in neurobiology

    2021  Volume 72, Page(s) 32–38

    Abstract: Here, we discuss findings made using Drosophila on Alzheimer's disease (AD) risk and progression. Recent studies have investigated the mechanisms underlying glia-mediated neuroprotection in AD. First, we discuss a novel mechanism of glial lipid droplet ... ...

    Abstract Here, we discuss findings made using Drosophila on Alzheimer's disease (AD) risk and progression. Recent studies have investigated the mechanisms underlying glia-mediated neuroprotection in AD. First, we discuss a novel mechanism of glial lipid droplet formation that occurs in response to elevated reactive oxygen species in neurons. The data suggest that disruptions to this process contribute to AD risk. We further discuss novel mechanistic insights into glia-mediated Aβ42-clearance made using the fly. Finally, we highlight work that provides evidence that the aberrant accumulation of reactive oxygen species in AD may not just be a consequence of disease but contribute to disease progression as well. Cumulatively, the discussed studies highlight recent, relevant discoveries in AD made using Drosophila.
    MeSH term(s) Alzheimer Disease ; Amyloid beta-Peptides/metabolism ; Animals ; Drosophila ; Neuroglia/metabolism ; Oxidative Stress
    Chemical Substances Amyloid beta-Peptides
    Language English
    Publishing date 2021-08-18
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1078046-4
    ISSN 1873-6882 ; 0959-4388
    ISSN (online) 1873-6882
    ISSN 0959-4388
    DOI 10.1016/j.conb.2021.07.012
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 3260: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

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  6. Article ; Online: Integrating non-mammalian model organisms in the diagnosis of rare genetic diseases in humans.

    Yamamoto, Shinya / Kanca, Oguz / Wangler, Michael F / Bellen, Hugo J

    Nature reviews. Genetics

    2023  Volume 25, Issue 1, Page(s) 46–60

    Abstract: Next-generation sequencing technology has rapidly accelerated the discovery of genetic variants of interest in individuals with rare diseases. However, showing that these variants are causative of the disease in question is complex and may require ... ...

    Abstract Next-generation sequencing technology has rapidly accelerated the discovery of genetic variants of interest in individuals with rare diseases. However, showing that these variants are causative of the disease in question is complex and may require functional studies. Use of non-mammalian model organisms - mainly fruitflies (Drosophila melanogaster), nematode worms (Caenorhabditis elegans) and zebrafish (Danio rerio) - enables the rapid and cost-effective assessment of the effects of gene variants, which can then be validated in mammalian model organisms such as mice and in human cells. By probing mechanisms of gene action and identifying interacting genes and proteins in vivo, recent studies in these non-mammalian model organisms have facilitated the diagnosis of numerous genetic diseases and have enabled the screening and identification of therapeutic options for patients. Studies in non-mammalian model organisms have also shown that the biological processes underlying rare diseases can provide insight into more common mechanisms of disease and the biological functions of genes. Here, we discuss the opportunities afforded by non-mammalian model organisms, focusing on flies, worms and fish, and provide examples of their use in the diagnosis of rare genetic diseases.
    MeSH term(s) Humans ; Animals ; Mice ; Rare Diseases ; Zebrafish/genetics ; Drosophila melanogaster/genetics ; Caenorhabditis elegans/genetics ; Mammals
    Language English
    Publishing date 2023-07-25
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2035157-4
    ISSN 1471-0064 ; 1471-0056
    ISSN (online) 1471-0064
    ISSN 1471-0056
    DOI 10.1038/s41576-023-00633-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 5474: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MG 40: Show issues

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  7. Article: Sphingolipids in neurodegenerative diseases.

    Pan, Xueyang / Dutta, Debdeep / Lu, Shenzhao / Bellen, Hugo J

    Frontiers in neuroscience

    2023  Volume 17, Page(s) 1137893

    Abstract: Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive deficits of neuronal function. Recent evidence argues that sphingolipid metabolism is affected in a surprisingly broad set of NDDs. These include some lysosomal storage ... ...

    Abstract Neurodegenerative Diseases (NDDs) are a group of disorders that cause progressive deficits of neuronal function. Recent evidence argues that sphingolipid metabolism is affected in a surprisingly broad set of NDDs. These include some lysosomal storage diseases (LSDs), hereditary sensory and autonomous neuropathy (HSAN), hereditary spastic paraplegia (HSP), infantile neuroaxonal dystrophy (INAD), Friedreich's ataxia (FRDA), as well as some forms of amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD). Many of these diseases have been modeled in
    Language English
    Publishing date 2023-02-16
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2411902-7
    ISSN 1662-453X ; 1662-4548
    ISSN (online) 1662-453X
    ISSN 1662-4548
    DOI 10.3389/fnins.2023.1137893
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Using

    Link, Nichole / Bellen, Hugo J

    Development (Cambridge, England)

    2020  Volume 147, Issue 21

    Abstract: Next-generation sequencing has greatly accelerated the discovery of rare human genetic diseases. Nearly 45% of patients have variants associated with known diseases but the unsolved cases remain a conundrum. Moreover, causative mutations can be difficult ...

    Abstract Next-generation sequencing has greatly accelerated the discovery of rare human genetic diseases. Nearly 45% of patients have variants associated with known diseases but the unsolved cases remain a conundrum. Moreover, causative mutations can be difficult to pinpoint because variants frequently map to genes with no previous disease associations and, often, only one or a few patients with variants in the same gene are identified. Model organisms, such as
    MeSH term(s) Animals ; Disease Models, Animal ; Drosophila melanogaster/genetics ; Genetic Techniques ; Human Genetics ; Humans ; Microcephaly/diagnosis ; Microcephaly/genetics ; Rare Diseases/diagnosis ; Rare Diseases/genetics
    Language English
    Publishing date 2020-09-28
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 90607-4
    ISSN 1477-9129 ; 0950-1991
    ISSN (online) 1477-9129
    ISSN 0950-1991
    DOI 10.1242/dev.191411
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Ub I Zs.183: Show issues Location:
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    Zs.MG 91: Show issues

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  9. Article: CK2α-dependent regulation of Wnt activity governs white matter development and repair.

    Wang, Chih-Yen / Zuo, Zhongyuan / Kim, Kyoung In / Bellen, Hugo J / Lee, Hyun Kyoung

    bioRxiv : the preprint server for biology

    2023  

    Abstract: Wnt signaling plays an essential role in developmental and regenerative myelination in the CNS. The Wnt signaling pathway is comprised of multiple regulatory layers; thus, how these processes are coordinated to orchestrate oligodendrocyte development ... ...

    Abstract Wnt signaling plays an essential role in developmental and regenerative myelination in the CNS. The Wnt signaling pathway is comprised of multiple regulatory layers; thus, how these processes are coordinated to orchestrate oligodendrocyte development remains unclear. Here we show CK2α, a Wnt/β-catenin signaling Ser/Thr kinase, phosphorylates Daam2, inhibiting its function and Wnt-activity during oligodendrocyte development. Intriguingly, we found Daam2 phosphorylation differentially impacts distinct stages of oligodendrocyte development, accelerating early differentiation followed by decelerating maturation and myelination. Application towards white matter injury revealed CK2α-mediated Daam2 phosphorylation plays a protective role for developmental and behavioral recovery after neonatal hypoxia, while promoting myelin repair following adult demyelination. Together, our findings identify a novel regulatory node in the Wnt pathway that regulates oligodendrocyte development via protein phosphorylation-induced signaling complex instability and highlights a new biological mechanism for myelin restoration.
    Significance: Wnt signaling plays a vital role in OL development and has been implicated as an adverse event for myelin repair after white matter injury. Emerging studies have shed light on multi-modal roles of Wnt effectors in the OL lineage, but the underlying molecular mechanisms and modifiable targets in OL remyelination remain unclear. Using genetic mouse development and injury model systems, we delineate a novel stage-specific function of Daam2 in Wnt signaling and OL development via a S704/T7-5 phosphorylation mechanism, and determine a new role of the kinase CK2α in contributing to OL development. In-depth understanding of CK2α-Daam2 pathway regulation will allow us to precisely modulate its activity in conjunction with Wnt signaling and harness its biology for white matter pathology.
    Language English
    Publishing date 2023-04-11
    Publishing country United States
    Document type Preprint
    DOI 10.1101/2023.04.11.536369
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: The Voltage-Gated Sodium Channel in

    Ravenscroft, Thomas A / Jacobs, Ashleigh / Gu, Mingxue / Eberl, Daniel F / Bellen, Hugo J

    eNeuro

    2023  Volume 10, Issue 6

    Abstract: The fruit ... ...

    Abstract The fruit fly
    MeSH term(s) Animals ; Action Potentials ; Axons/metabolism ; Dendrites/metabolism ; Drosophila ; Drosophila melanogaster/physiology ; Sensory Receptor Cells/metabolism ; Transient Receptor Potential Channels/metabolism ; Voltage-Gated Sodium Channels
    Chemical Substances Transient Receptor Potential Channels ; Voltage-Gated Sodium Channels ; para protein, Drosophila
    Language English
    Publishing date 2023-06-29
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2800598-3
    ISSN 2373-2822 ; 2373-2822
    ISSN (online) 2373-2822
    ISSN 2373-2822
    DOI 10.1523/ENEURO.0105-23.2023
    Database MEDical Literature Analysis and Retrieval System OnLINE

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