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  1. Book: Neurodegeneration

    Manfredi, Giovanni / Kawamata, Hibiki

    methods and protocols

    (Methods in molecular biology ; 793 ; Springer protocols)

    2011  

    Author's details ed. by Giovanni Manfredi ; Hibiki Kawamata
    Series title Methods in molecular biology ; 793
    Springer protocols
    Collection
    Language English
    Size XV, 512 S. : Ill., graph. Darst.
    Publisher Humana Press
    Publishing place New York
    Publishing country United States
    Document type Book
    HBZ-ID HT016963511
    ISBN 978-1-61779-327-1 ; 1-61779-327-2
    Database Catalogue ZB MED Medicine, Health

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    Shelf markLoan statusLocation
    2011 A 4152 order for loan Magazin

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  2. Article ; Online: Correction: Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.

    Kawamata, Hibiki / Manfredi, Giovanni

    The Journal of cell biology

    2017  Volume 217, Issue 1, Page(s) 429

    Language English
    Publishing date 2017-12-21
    Publishing country United States
    Document type Journal Article ; Published Erratum
    ZDB-ID 218154-x
    ISSN 1540-8140 ; 0021-9525
    ISSN (online) 1540-8140
    ISSN 0021-9525
    DOI 10.1083/JCB.20170917212192017c
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

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  3. Article ; Online: Glial mitochondrial function and dysfunction in health and neurodegeneration.

    McAvoy, Kevin / Kawamata, Hibiki

    Molecular and cellular neurosciences

    2019  Volume 101, Page(s) 103417

    Abstract: Mitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases, mutations that impair specific oxidative phosphorylation (OXPHOS) proteins or OXPHOS assembly ... ...

    Abstract Mitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases, mutations that impair specific oxidative phosphorylation (OXPHOS) proteins or OXPHOS assembly factors lead to isolated biochemical defects and a heterogeneous group of clinical phenotypes, including mitochondrial encephalopathies. A broader defect of OXPHOS function, due to mutations in proteins involved in mitochondrial DNA maintenance, mitochondrial biogenesis, or mitochondrial tRNAs can also underlie severe mitochondrial encephalopathies. While primary mitochondrial dysfunction causes rare genetic forms of neurological disorders, secondary mitochondrial dysfunction is involved in the pathophysiology of some of the most common neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis. Many studies have investigated mitochondrial function and dysfunction in bulk central nervous system (CNS) tissue. However, the interpretation of these studies has been often complicated by the extreme cellular heterogeneity of the CNS, which includes many different types of neurons and glial cells. Because neurons are especially dependent on OXPHOS for ATP generation, mitochondrial dysfunction is thought to be directly involved in cell autonomous neuronal demise. Despite being metabolically more flexible than neurons, glial mitochondria also play an essential role in the function of the CNS, and have adapted specific metabolic and mitochondrial features to support their diversity of functions. This review analyzes our current understanding and the gaps in knowledge of mitochondrial properties of glia and how they affect neuronal functions, in health and disease.
    MeSH term(s) Animals ; Astrocytes/metabolism ; Astrocytes/pathology ; Electron Transport Complex I/genetics ; Electron Transport Complex I/metabolism ; Humans ; Mitochondria/metabolism ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/metabolism ; Oxidative Phosphorylation
    Chemical Substances Electron Transport Complex I (EC 7.1.1.2)
    Language English
    Publishing date 2019-10-31
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ZDB-ID 1046640-x
    ISSN 1095-9327 ; 1044-7431
    ISSN (online) 1095-9327
    ISSN 1044-7431
    DOI 10.1016/j.mcn.2019.103417
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

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  4. Article: High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice.

    Southwell, Nneka / Manzo, Onorina / Zhao, Dazhi / Sayles, Nicole M / Dash, Jalia / Fujita, Keigo / D'Aurelio, Marilena / Di Lorenzo, Annarita / Manfredi, Giovanni / Kawamata, Hibiki

    bioRxiv : the preprint server for biology

    2023  

    Abstract: Mutations ... ...

    Abstract Mutations in
    Language English
    Publishing date 2023-12-28
    Publishing country United States
    Document type Preprint
    DOI 10.1101/2023.02.22.529577
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Effects of PB-TURSO on the transcriptional and metabolic landscape of sporadic ALS fibroblasts.

    Fels, Jasmine A / Dash, Jalia / Leslie, Kent / Manfredi, Giovanni / Kawamata, Hibiki

    Annals of clinical and translational neurology

    2022  Volume 9, Issue 10, Page(s) 1551–1564

    Abstract: Objective: ALS is a rapidly progressive, fatal disorder caused by motor neuron degeneration, for which there is a great unmet therapeutic need. AMX0035, a combination of sodium phenylbutyrate (PB) and taurursodiol (TUDCA, TURSO), has shown promising ... ...

    Abstract Objective: ALS is a rapidly progressive, fatal disorder caused by motor neuron degeneration, for which there is a great unmet therapeutic need. AMX0035, a combination of sodium phenylbutyrate (PB) and taurursodiol (TUDCA, TURSO), has shown promising results in early ALS clinical trials, but its mechanisms of action remain to be elucidated. Therefore, our goal was to obtain an unbiased landscape of the molecular effects of AMX0035 in ALS patient-derived cells.
    Methods: We investigated the transcriptomic and metabolomic profiles of primary skin fibroblasts from sporadic ALS patients and healthy controls (n = 12/group) treated with PB, TUDCA, or PB-TUDCA combination (Combo). Data were evaluated with multiple approaches including differential gene expression and metabolite abundance, Gene Ontology and metabolic pathway analysis, weighted gene co-expression correlation analysis (WGCNA), and combined multiomics integrated analysis.
    Results: Combo changed many more genes and metabolites than either PB or TUDCA individually. Most changes were unique to Combo and affected the expression of genes involved in nucleocytoplasmic transport, unfolded protein response, mitochondrial function, RNA metabolism, and innate immunity. WGCNA showed significant correlations between ALS gene expression modules and clinical parameters that were abolished by Combo treatment.
    Interpretation: This study is the first to explore the molecular effects of Combo in ALS patient-derived cells. It shows that Combo has a greater and distinct impact compared with the individual compounds and provides clues to drug targets and mechanisms of action, which may underlie the benefits of this investigational drug combination.
    MeSH term(s) Amyotrophic Lateral Sclerosis/drug therapy ; Amyotrophic Lateral Sclerosis/genetics ; Amyotrophic Lateral Sclerosis/metabolism ; Drugs, Investigational ; Fibroblasts/metabolism ; Humans ; RNA ; Taurochenodeoxycholic Acid
    Chemical Substances Drugs, Investigational ; Taurochenodeoxycholic Acid (516-35-8) ; ursodoxicoltaurine (60EUX8MN5X) ; RNA (63231-63-0)
    Language English
    Publishing date 2022-09-09
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
    ZDB-ID 2740696-9
    ISSN 2328-9503 ; 2328-9503
    ISSN (online) 2328-9503
    ISSN 2328-9503
    DOI 10.1002/acn3.51648
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.

    Kawamata, Hibiki / Manfredi, Giovanni

    The Journal of cell biology

    2017  Volume 216, Issue 12, Page(s) 3917–3929

    Abstract: Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major neurodegenerative diseases are characterized pathologically by accumulation of misfolded proteins ... ...

    Abstract Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major neurodegenerative diseases are characterized pathologically by accumulation of misfolded proteins as a result of gene mutations or abnormal protein homeostasis. Misfolded proteins associate with mitochondria, forming oligomeric and fibrillary aggregates. As mitochondrial dysfunction, particularly of the oxidative phosphorylation system (OXPHOS), occurs in neurodegeneration, it is postulated that such defects are caused by the accumulation of misfolded proteins. However, this hypothesis and the pathological role of proteinopathies in mitochondria remain elusive. In this study, we critically review the proposed mechanisms whereby exemplary misfolded proteins associate with mitochondria and their consequences on OXPHOS.
    MeSH term(s) Amyloid beta-Peptides/genetics ; Amyloid beta-Peptides/metabolism ; Animals ; Gene Expression Regulation ; Humans ; Mitochondria/genetics ; Mitochondria/metabolism ; Mitochondrial Proteins/genetics ; Mitochondrial Proteins/metabolism ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/metabolism ; Neurodegenerative Diseases/pathology ; Oxidative Phosphorylation ; Protein Interaction Mapping ; Proteostasis Deficiencies/genetics ; Proteostasis Deficiencies/metabolism ; Proteostasis Deficiencies/pathology ; alpha-Synuclein/genetics ; alpha-Synuclein/metabolism
    Chemical Substances Amyloid beta-Peptides ; Mitochondrial Proteins ; alpha-Synuclein
    Language English
    Publishing date 2017-11-22
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 218154-x
    ISSN 1540-8140 ; 0021-9525
    ISSN (online) 1540-8140
    ISSN 0021-9525
    DOI 10.1083/jcb.201709172
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Ub I Zs.190: Show issues Location:
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  7. Article ; Online: Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.

    Manfredi, Giovanni / Kawamata, Hibiki

    Neurobiology of disease

    2016  Volume 90, Page(s) 35–42

    Abstract: Physical and functional interactions between mitochondria and the endoplasmic reticulum (ER) are crucial for cell life. These two organelles are intimately connected and collaborate to essential processes, such as calcium homeostasis and phospholipid ... ...

    Abstract Physical and functional interactions between mitochondria and the endoplasmic reticulum (ER) are crucial for cell life. These two organelles are intimately connected and collaborate to essential processes, such as calcium homeostasis and phospholipid biosynthesis. The connections between mitochondria and endoplasmic reticulum occur through structures named mitochondria associated membranes (MAMs), which contain lipid rafts and a large number of proteins, many of which serve multiple functions at different cellular sites. Growing evidence strongly suggests that alterations of ER-mitochondria interactions are involved in neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), a devastating and rapidly fatal motor neuron disease. Mutations in proteins that participate in ER-mitochondria interactions and MAM functions are increasingly being associated with genetic forms of ALS and other neurodegenerative diseases. This evidence strongly suggests that, rather than considering the two organelles separately, a better understanding of the disease process can derive from studying the alterations in their crosstalk. In this review we discuss normal and pathological ER-mitochondria interactions and the evidence that link them to ALS.
    MeSH term(s) Amyotrophic Lateral Sclerosis/metabolism ; Animals ; Endoplasmic Reticulum/metabolism ; Humans ; Mitochondria/metabolism
    Language English
    Publishing date 2016-06
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1211786-9
    ISSN 1095-953X ; 0969-9961
    ISSN (online) 1095-953X
    ISSN 0969-9961
    DOI 10.1016/j.nbd.2015.08.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

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  8. Article ; Online: Nrf2 signaling links ER oxidative protein folding and calcium homeostasis in health and disease.

    Granatiero, Veronica / Konrad, Csaba / Bredvik, Kirsten / Manfredi, Giovanni / Kawamata, Hibiki

    Life science alliance

    2019  Volume 2, Issue 5

    Abstract: We report a signaling pathway linking two fundamental functions of the ER, oxidative protein folding, and intracellular calcium regulation. Cells sense ER oxidative protein folding through ... ...

    Abstract We report a signaling pathway linking two fundamental functions of the ER, oxidative protein folding, and intracellular calcium regulation. Cells sense ER oxidative protein folding through H
    MeSH term(s) Animals ; COS Cells ; Calcium/metabolism ; Cells, Cultured ; Chlorocebus aethiops ; Endoplasmic Reticulum/metabolism ; Endoplasmic Reticulum Stress ; HeLa Cells ; Homeostasis ; Humans ; Hydrogen Peroxide/pharmacology ; Mice ; Motor Neurons/cytology ; Motor Neurons/drug effects ; Motor Neurons/metabolism ; NF-E2-Related Factor 2/metabolism ; Peroxidases/metabolism ; Signal Transduction/drug effects
    Chemical Substances NF-E2-Related Factor 2 ; Hydrogen Peroxide (BBX060AN9V) ; GPX8 protein, human (EC 1.11.1.-) ; Peroxidases (EC 1.11.1.-) ; Calcium (SY7Q814VUP)
    Language English
    Publishing date 2019-10-28
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ISSN 2575-1077
    ISSN (online) 2575-1077
    DOI 10.26508/lsa.201900563
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy.

    Sayles, Nicole M / Southwell, Nneka / McAvoy, Kevin / Kim, Kihwan / Pesini, Alba / Anderson, Corey J / Quinzii, Catarina / Cloonan, Suzanne / Kawamata, Hibiki / Manfredi, Giovanni

    Cell reports

    2022  Volume 38, Issue 10, Page(s) 110475

    Abstract: Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of heart mitochondrial function activate the mitochondrial integrated stress response ( ... ...

    Abstract Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of heart mitochondrial function activate the mitochondrial integrated stress response (ISR
    MeSH term(s) Animals ; Cardiomyopathies/pathology ; Disease Models, Animal ; Mice ; Mitochondria/metabolism ; Mitochondrial Diseases/metabolism ; Mitochondrial Proteins/genetics ; Mitochondrial Proteins/metabolism
    Chemical Substances Mitochondrial Proteins
    Language English
    Publishing date 2022-03-09
    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.2022.110475
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Book: Neurodegeneration

    Manfredi, Giovanni / Kawamata, Hibiki

    methods and protocols

    (Methods in molecular biology, ; 793 ; Springer protocols)

    2011  

    Title variant Neuro-degeneration
    Author's details edited by Giovanni Manfredi, Hibiki Kawamata
    Series title Methods in molecular biology, ; 793
    Springer protocols
    MeSH term(s) Nerve Degeneration ; Cell Culture Techniques/methods ; Neurodegenerative Diseases/physiopathology ; Disease Models, Animal
    Language English
    Size xv, 512 p. :, ill. ;, 26 cm.
    Publisher Humana Press
    Publishing place New York, NY
    Document type Book
    ISBN 9781617793271 ; 1617793272 ; 9781617793288 ; 1617793280
    Database Catalogue of the US National Library of Medicine (NLM)

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