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  1. AU="Quarta, Raffaella"
  2. AU="Samuel Fernández-Carnero"
  3. AU="Kastner, Brigitte"
  4. AU=Robins Harlan
  5. AU="Sundaram, Baskaran"
  6. AU=Whitby R L D
  7. AU="Michel M. Santos"
  8. AU=Chong Yong-Yeow
  9. AU="Posca-Maina, Marcela"
  10. AU="Trupin, Laura"
  11. AU="Bezerra, Antônio Diego M."
  12. AU="Xiao-hua Lin"
  13. AU="Ksenia Zaytseva" AU="Ksenia Zaytseva"
  14. AU="Michael N Alexis"
  15. AU="Rivero-Moreno, Yeisson"
  16. AU="Köllmann, Nienke"
  17. AU="Maveddat, Ashley"
  18. AU=Boerrigter Guido
  19. AU=Wajant H
  20. AU=Marsboom Glenn
  21. AU="Xuwei, Tao"
  22. AU="Matias, Ricardo"
  23. AU="Daly, Brian P."
  24. AU="Bissaro, Maicol"
  25. AU="Mateo, Mathieu"
  26. AU="Yousra Aouinati"
  27. AU=Butros Linda
  28. AU=CASTORENA-GONZALEZ JORGE A.
  29. AU=Grtsch Bettina
  30. AU="José M. Ramada"
  31. AU="Parashar, Prashant"

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  1. Artikel ; Online: Ion channels as biomarkers of altered myogenesis in myofiber precursors of Duchenne muscular dystrophy.

    Cerchiara, Alessandro Giovanni / Imbrici, Paola / Quarta, Raffaella / Cristiano, Enrica / Boccanegra, Brigida / Caputo, Erika / Wells, Dominic J / Cappellari, Ornella / De Luca, Annamaria

    Annals of the New York Academy of Sciences

    2024  Band 1534, Heft 1, Seite(n) 130–144

    Abstract: Myogenesis is essential for skeletal muscle formation, growth, and regeneration and can be altered in Duchenne muscular dystrophy (DMD), an X-linked disorder due to the absence of the cytoskeletal protein dystrophin. Ion channels play a pivotal role in ... ...

    Abstract Myogenesis is essential for skeletal muscle formation, growth, and regeneration and can be altered in Duchenne muscular dystrophy (DMD), an X-linked disorder due to the absence of the cytoskeletal protein dystrophin. Ion channels play a pivotal role in muscle differentiation and interact with the dystrophin complex. To investigate ion channel involvement in myogenesis in dystrophic settings, we performed electrophysiological characterization of two immortalized mouse cell lines, wild-type (WT) H2K-2B4 and the dystrophic (DYS) H2K-SF1, and measured gene expression of differentiation markers and ion channels. Inward and outward currents/density increased as differentiation progressed in both WT and DYS cells. However, day-11 DYS cells showed higher (27%) inward current density with an increased expression ratio of Scn5a/Scn4a and decreased (48%) barium-sensitive outward current compared to WT. Furthermore, day-11 DYS cells showed more positive resting membrane potential (+10 mV) and lower membrane capacitance (50%) compared to WT. DYS cells also had reduced Myog and Myf5 expression at days 6 and 11. Overall, ion channel profile and myogenesis appeared altered in DYS cells. These results are a first step in validating ion channels as potential drug targets to ameliorate muscle degeneration in DMD settings and as differentiation biomarkers in innovative platforms.
    Mesh-Begriff(e) Animals ; Mice ; Muscular Dystrophy, Duchenne/metabolism ; Dystrophin/metabolism ; Muscle, Skeletal/metabolism ; Biomarkers/metabolism ; Ion Channels/metabolism ; Muscle Development
    Chemische Substanzen Dystrophin ; Biomarkers ; Ion Channels
    Sprache Englisch
    Erscheinungsdatum 2024-03-22
    Erscheinungsland United States
    Dokumenttyp Journal Article
    ZDB-ID 211003-9
    ISSN 1749-6632 ; 0077-8923
    ISSN (online) 1749-6632
    ISSN 0077-8923
    DOI 10.1111/nyas.15124
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  2. Artikel ; Online: LKB1 signaling is altered in skeletal muscle of a Duchenne muscular dystrophy mouse model.

    Boccanegra, Brigida / Mantuano, Paola / Conte, Elena / Cerchiara, Alessandro Giovanni / Tulimiero, Lisamaura / Quarta, Raffaella / Caputo, Erika / Sanarica, Francesca / Forino, Monica / Spadotto, Valeria / Cappellari, Ornella / Fossati, Gianluca / Steinkühler, Christian / De Luca, Annamaria

    Disease models & mechanisms

    2023  Band 16, Heft 7

    Abstract: The potential role of liver kinase B1 (LKB1) in the altered activation of the master metabolic and epigenetic regulator adenosine monophosphate-activated protein kinase (AMPK) in Duchenne muscular dystrophy has not been investigated so far. Hence, we ... ...

    Abstract The potential role of liver kinase B1 (LKB1) in the altered activation of the master metabolic and epigenetic regulator adenosine monophosphate-activated protein kinase (AMPK) in Duchenne muscular dystrophy has not been investigated so far. Hence, we analyzed both gene and protein levels of LKB1 and its related targets in gastrocnemius muscles of adult C57BL/10 mdx mice and D2 mdx mice, a model with a more severe dystrophic phenotype, as well as the sensitivity of the LKB1-AMPK pathway to AMPK activators, such as chronic exercise. Our data show, for the first time, a reduction in the levels of LKB1 and accessory proteins, MO25 and STRADα, in both mdx strains versus the respective wild type, which was further impaired by exercise, in parallel with a lack of further phosphorylation of AMPK. The AMPK-like kinase salt-inducible kinase (SIK) and class II histone deacetylases, along with expression of the HDAC target gene Mef2c, were also altered, supporting an impairment of LKB1-SIK-class II histone deacetylase signaling. Our results demonstrate that LKB1 may be involved in dystrophic progression, paving the way for future preclinical studies.
    Mesh-Begriff(e) Animals ; Mice ; AMP-Activated Protein Kinases/metabolism ; Disease Models, Animal ; Mice, Inbred C57BL ; Mice, Inbred mdx ; Muscle, Skeletal/metabolism ; Muscular Dystrophy, Duchenne/genetics ; Muscular Dystrophy, Duchenne/metabolism ; Protein Serine-Threonine Kinases/metabolism
    Chemische Substanzen AMP-Activated Protein Kinases (EC 2.7.11.31) ; Protein Serine-Threonine Kinases (EC 2.7.11.1) ; Stk11 protein, mouse (EC 2.7.11.1)
    Sprache Englisch
    Erscheinungsdatum 2023-07-10
    Erscheinungsland England
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2451104-3
    ISSN 1754-8411 ; 1754-8403
    ISSN (online) 1754-8411
    ISSN 1754-8403
    DOI 10.1242/dmm.049930
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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  3. Artikel ; Online: DTI and PWI analysis of peri-enhancing tumoral brain tissue in patients treated for glioblastoma.

    Stecco, Alessandro / Pisani, Carla / Quarta, Raffaella / Brambilla, Marco / Masini, Laura / Beldì, Debora / Zizzari, Sara / Fossaceca, Rita / Krengli, Marco / Carriero, Alessandro

    Journal of neuro-oncology

    2010  Band 102, Heft 2, Seite(n) 261–271

    Abstract: To analyse the role of MR diffusion-tensor imaging (DTI) and perfusion-weighted imaging (PWI) in characterising tumour boundaries in patients with glioblastoma multiforme. Seventeen patients with surgically treated WHO IV grade gliomas who were ... ...

    Abstract To analyse the role of MR diffusion-tensor imaging (DTI) and perfusion-weighted imaging (PWI) in characterising tumour boundaries in patients with glioblastoma multiforme. Seventeen patients with surgically treated WHO IV grade gliomas who were candidates for adjuvant chemo-radiotherapy were enrolled. Before (T0) and after radiation treatment (T1), they underwent DTI and PWI, and the apparent diffusion coefficient (ADC), fractional anisotropy (FA) and relative cerebral blood volume (rCBV) in the enhancing tumour, the hyperintense tissue adjacent to the enhancing tumour, and the normal-appearing white matter (NAWM) adjacent to the hyperintense areas were analysed. The enhancing tissue at T1 was retrospectively divided on the basis of whether or not it was also enhancing at T0. The controls were the corresponding contralateral areas, on which we normalized the rCBV values, calculating the rCBV ratio. In NAWM, we did not find any significant differences in FA, ADC or rCBV. In the hyperintense perilesional regions, FA was significantly lower and ADC significantly higher than in the unaffected contralateral tissue; there were no significant differences in the rCBV maps. The values of FA, ADC and rCBV in enhancing neoplastic tissue were all significantly different from those observed in the contralateral tissue. There was no significant difference in rCBV values between the areas enhancing at T0 and those not enhancing at T0 but enhancing at T1, which may indicate the neoplastic transformation of apparently normal brain tissue. DTI metrics identify ultrastructural changes in hyperintense perilesional areas, but these are not specific for neoplastic tissue. rCBV seemed to reflect an ultrastructural alteration that was not visible at T0, but became visible (as neoplastic progression) on conventional MR images at T1. These findings could help identify tissue at risk of tumour infiltration.
    Mesh-Begriff(e) Anisotropy ; Blood Volume ; Brain Neoplasms/pathology ; Brain Neoplasms/therapy ; Diffusion Tensor Imaging ; Glioblastoma/pathology ; Glioblastoma/therapy ; Humans ; Image Processing, Computer-Assisted ; Perfusion Imaging ; Retrospective Studies
    Sprache Englisch
    Erscheinungsdatum 2010-07-25
    Erscheinungsland United States
    Dokumenttyp Journal Article
    ZDB-ID 604875-4
    ISSN 1573-7373 ; 0167-594X
    ISSN (online) 1573-7373
    ISSN 0167-594X
    DOI 10.1007/s11060-010-0310-x
    Datenquelle MEDical Literature Analysis and Retrieval System OnLINE

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