Article

 

Muscle stem cell dysfunction impairs muscle regeneration in a mouse model of Down syndrome. Öffentlichkeit Deposited

https://scholar.colorado.edu/concern/articles/ks65hc85n
Abstract
  • Down syndrome, caused by trisomy 21, is characterized by a variety of medical conditions including intellectual impairments, cardiovascular defects, blood cell disorders and pre-mature aging phenotypes. Several somatic stem cell populations are dysfunctional in Down syndrome and their deficiencies may contribute to multiple Down syndrome phenotypes. Down syndrome is associated with muscle weakness but skeletal muscle stem cells or satellite cells in Down syndrome have not been investigated. We find that a failure in satellite cell expansion impairs muscle regeneration in the Ts65Dn mouse model of Down syndrome. Ts65Dn satellite cells accumulate DNA damage and over express Usp16, a histone de-ubiquitinating enzyme that regulates the DNA damage response. Impairment of satellite cell function, which further declines as Ts65Dn mice age, underscores stem cell deficiencies as an important contributor to Down syndrome pathologies.
Creator
Date Issued
  • 2018-03-09
Academic Affiliation
Journal Title
Journal Issue/Number
  • 1
Journal Volume
  • 8
File Extent
  • 4309-4309
Zuletzt geändert
  • 2019-12-05
Identifier
  • PubMed ID: 29523805
Resource Type
Urheberrechts-Erklärung
DOI
ISSN
  • 2045-2322
Language
License

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