View in EDS HTML Full Text PDF Full Text

Abnormal nuclear aggregation and myotube degeneration in myotonic dystrophy type 1.

Saved in:
Bibliographic Details
Title: Abnormal nuclear aggregation and myotube degeneration in myotonic dystrophy type 1.
Authors: Wang, Yanlin (AUTHOR), Hao, Lei (AUTHOR), Li, Hui (AUTHOR), Cleary, John D. (AUTHOR), Tomac, Michael P. (AUTHOR), Thapa, Arjun (AUTHOR), Guo, Xiuming (AUTHOR), Zeng, Desmond (AUTHOR), Wang, Hongcai (AUTHOR), McRae, MacKezie (AUTHOR), Jastrzemski, Olivia (AUTHOR), Smith-Fassler, Ali Marichen (AUTHOR), Xu, Yuming (AUTHOR), Xia, Guangbin (AUTHOR)
Source: Neurological Sciences. Jun2019, Vol. 40 Issue 6, p1255-1265. 11p. 6 Color Photographs, 2 Black and White Photographs, 3 Graphs.
Subjects: Myotonia atrophica, Degeneration (Pathology), Protein kinases, Genome editing, Muscle regeneration, Skeletal muscle physiology, In vitro studies, Skeletal muscle, Research funding, Cell physiology, Regeneration (Biology), Cell culture, Cell nuclei, Animal experimentation, Stem cells
Abstract: Myotonic dystrophy type 1 (DM1) is caused by CTG nucleotide repeat expansions in the 3'-untranslated region (3'-UTR) of the dystrophia myotonica protein kinase (DMPK) gene. The expanded CTG repeats encode toxic CUG RNAs that cause disease, largely through RNA gain-of-function. DM1 is a fatal disease characterized by progressive muscle wasting, which has no cure. Regenerative medicine has emerged as a promising therapeutic modality for DM1, especially with the advancement of induced pluripotent stem (iPS) cell technology and therapeutic genome editing. However, there is an unmet need to identify in vitro outcome measures to demonstrate the therapeutic effects prior to in vivo clinical trials. In this study, we examined the muscle regeneration (myotube formation) in normal and DM1 myoblasts in vitro to establish outcome measures for therapeutic monitoring. We found normal proliferation of DM1 myoblasts, but abnormal nuclear aggregation during the early stage myotube formation, as well as myotube degeneration during the late stage of myotube formation. We concluded that early abnormal nuclear aggregation and late myotube degeneration offer easy and sensitive outcome measures to monitor therapeutic effects in vitro. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
Full text is not displayed to guests.
FullText Links:
  – Type: pdflink
Text:
  Availability: 1
Header DbId: pbh
DbLabel: Psychology and Behavioral Sciences Collection
An: 136540475
AccessLevel: 6
PubType: Academic Journal
PubTypeId: academicJournal
PreciseRelevancyScore: 0
IllustrationInfo
Items – Name: Title
  Label: Title
  Group: Ti
  Data: Abnormal nuclear aggregation and myotube degeneration in myotonic dystrophy type 1.
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22Wang%2C+Yanlin%22">Wang, Yanlin</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Hao%2C+Lei%22">Hao, Lei</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Li%2C+Hui%22">Li, Hui</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Cleary%2C+John+D%2E%22">Cleary, John D.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Tomac%2C+Michael+P%2E%22">Tomac, Michael P.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Thapa%2C+Arjun%22">Thapa, Arjun</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Guo%2C+Xiuming%22">Guo, Xiuming</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Zeng%2C+Desmond%22">Zeng, Desmond</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Wang%2C+Hongcai%22">Wang, Hongcai</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22McRae%2C+MacKezie%22">McRae, MacKezie</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Jastrzemski%2C+Olivia%22">Jastrzemski, Olivia</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Smith-Fassler%2C+Ali+Marichen%22">Smith-Fassler, Ali Marichen</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Xu%2C+Yuming%22">Xu, Yuming</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Xia%2C+Guangbin%22">Xia, Guangbin</searchLink> (AUTHOR)
– Name: TitleSource
  Label: Source
  Group: Src
  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. Jun2019, Vol. 40 Issue 6, p1255-1265. 11p. 6 Color Photographs, 2 Black and White Photographs, 3 Graphs.
– Name: Subject
  Label: Subjects
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22Myotonia+atrophica%22">Myotonia atrophica</searchLink><br /><searchLink fieldCode="DE" term="%22Degeneration+%28Pathology%29%22">Degeneration (Pathology)</searchLink><br /><searchLink fieldCode="DE" term="%22Protein+kinases%22">Protein kinases</searchLink><br /><searchLink fieldCode="DE" term="%22Genome+editing%22">Genome editing</searchLink><br /><searchLink fieldCode="DE" term="%22Muscle+regeneration%22">Muscle regeneration</searchLink><br /><searchLink fieldCode="DE" term="%22Skeletal+muscle+physiology%22">Skeletal muscle physiology</searchLink><br /><searchLink fieldCode="DE" term="%22In+vitro+studies%22">In vitro studies</searchLink><br /><searchLink fieldCode="DE" term="%22Skeletal+muscle%22">Skeletal muscle</searchLink><br /><searchLink fieldCode="DE" term="%22Research+funding%22">Research funding</searchLink><br /><searchLink fieldCode="DE" term="%22Cell+physiology%22">Cell physiology</searchLink><br /><searchLink fieldCode="DE" term="%22Regeneration+%28Biology%29%22">Regeneration (Biology)</searchLink><br /><searchLink fieldCode="DE" term="%22Cell+culture%22">Cell culture</searchLink><br /><searchLink fieldCode="DE" term="%22Cell+nuclei%22">Cell nuclei</searchLink><br /><searchLink fieldCode="DE" term="%22Animal+experimentation%22">Animal experimentation</searchLink><br /><searchLink fieldCode="DE" term="%22Stem+cells%22">Stem cells</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Myotonic dystrophy type 1 (DM1) is caused by CTG nucleotide repeat expansions in the 3'-untranslated region (3'-UTR) of the dystrophia myotonica protein kinase (DMPK) gene. The expanded CTG repeats encode toxic CUG RNAs that cause disease, largely through RNA gain-of-function. DM1 is a fatal disease characterized by progressive muscle wasting, which has no cure. Regenerative medicine has emerged as a promising therapeutic modality for DM1, especially with the advancement of induced pluripotent stem (iPS) cell technology and therapeutic genome editing. However, there is an unmet need to identify in vitro outcome measures to demonstrate the therapeutic effects prior to in vivo clinical trials. In this study, we examined the muscle regeneration (myotube formation) in normal and DM1 myoblasts in vitro to establish outcome measures for therapeutic monitoring. We found normal proliferation of DM1 myoblasts, but abnormal nuclear aggregation during the early stage myotube formation, as well as myotube degeneration during the late stage of myotube formation. We concluded that early abnormal nuclear aggregation and late myotube degeneration offer easy and sensitive outcome measures to monitor therapeutic effects in vitro. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
PLink https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=pbh&AN=136540475
RecordInfo BibRecord:
  BibEntity:
    Identifiers:
      – Type: doi
        Value: 10.1007/s10072-019-03783-w
    Languages:
      – Code: eng
        Text: English
    PhysicalDescription:
      Pagination:
        PageCount: 11
        StartPage: 1255
    Subjects:
      – SubjectFull: Myotonia atrophica
        Type: general
      – SubjectFull: Degeneration (Pathology)
        Type: general
      – SubjectFull: Protein kinases
        Type: general
      – SubjectFull: Genome editing
        Type: general
      – SubjectFull: Muscle regeneration
        Type: general
      – SubjectFull: Skeletal muscle physiology
        Type: general
      – SubjectFull: In vitro studies
        Type: general
      – SubjectFull: Skeletal muscle
        Type: general
      – SubjectFull: Research funding
        Type: general
      – SubjectFull: Cell physiology
        Type: general
      – SubjectFull: Regeneration (Biology)
        Type: general
      – SubjectFull: Cell culture
        Type: general
      – SubjectFull: Cell nuclei
        Type: general
      – SubjectFull: Animal experimentation
        Type: general
      – SubjectFull: Stem cells
        Type: general
    Titles:
      – TitleFull: Abnormal nuclear aggregation and myotube degeneration in myotonic dystrophy type 1.
        Type: main
  BibRelationships:
    HasContributorRelationships:
      – PersonEntity:
          Name:
            NameFull: Wang, Yanlin
      – PersonEntity:
          Name:
            NameFull: Hao, Lei
      – PersonEntity:
          Name:
            NameFull: Li, Hui
      – PersonEntity:
          Name:
            NameFull: Cleary, John D.
      – PersonEntity:
          Name:
            NameFull: Tomac, Michael P.
      – PersonEntity:
          Name:
            NameFull: Thapa, Arjun
      – PersonEntity:
          Name:
            NameFull: Guo, Xiuming
      – PersonEntity:
          Name:
            NameFull: Zeng, Desmond
      – PersonEntity:
          Name:
            NameFull: Wang, Hongcai
      – PersonEntity:
          Name:
            NameFull: McRae, MacKezie
      – PersonEntity:
          Name:
            NameFull: Jastrzemski, Olivia
      – PersonEntity:
          Name:
            NameFull: Smith-Fassler, Ali Marichen
      – PersonEntity:
          Name:
            NameFull: Xu, Yuming
      – PersonEntity:
          Name:
            NameFull: Xia, Guangbin
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 01
              M: 06
              Text: Jun2019
              Type: published
              Y: 2019
          Identifiers:
            – Type: issn-print
              Value: 15901874
          Numbering:
            – Type: volume
              Value: 40
            – Type: issue
              Value: 6
          Titles:
            – TitleFull: Neurological Sciences
              Type: main
ResultId 1