Related Experiment Video

Updated: Feb 13, 2026

Examining Muscle Regeneration in Zebrafish Models of Muscle Disease
07:58

Examining Muscle Regeneration in Zebrafish Models of Muscle Disease

Published on: January 18, 2021

5.7K

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

Bradley Pawlikowski1,2, Nicole Dalla Betta1,2, Tiffany Elston1,2

  • 1Department of Molecular, Cellular and Developmental Biology, University of Colorado, 347 UCB, Boulder, CO, 80309, United States.

Scientific Reports
|March 11, 2018
PubMed

Insights

Satellite cells, crucial for muscle repair, show impaired expansion and increased DNA damage in Down syndrome models. This dysfunction contributes to muscle weakness and aging phenotypes in Down syndrome.

Area of Science:

  • Genetics and Molecular Biology
  • Stem Cell Biology
  • Developmental Biology

Background:

  • Down syndrome (DS), caused by trisomy 21, presents with intellectual disability, congenital heart defects, and premature aging.
  • Somatic stem cell dysfunction is implicated in various DS phenotypes.
  • Skeletal muscle stem cells (satellite cells) in DS have not been previously studied.

Purpose of the Study:

  • To investigate the function of satellite cells in a mouse model of Down syndrome.
  • To determine if satellite cell dysfunction contributes to muscle weakness observed in Down syndrome.

Main Methods:

  • Utilized the Ts65Dn mouse model, a genetic model for Down syndrome.
  • Assessed satellite cell expansion and muscle regeneration capacity.
  • Analyzed DNA damage accumulation and Usp16 expression in Ts65Dn satellite cells.

Main Results:

  • Ts65Dn satellite cells exhibited impaired expansion, hindering muscle regeneration.
  • Accumulation of DNA damage was observed in Ts65Dn satellite cells.
  • Overexpression of Usp16, a regulator of DNA damage response, was detected in these cells.
  • Satellite cell function further declined with age in the Ts65Dn mice.

Conclusions:

  • Satellite cell dysfunction is a significant contributor to muscle regeneration deficits in Down syndrome.
  • Accumulated DNA damage and altered Usp16 expression underlie satellite cell impairment in DS.
  • Stem cell deficiencies are key factors in the pathogenesis of Down syndrome phenotypes.

More Related Videos

Transplantation of Induced Pluripotent Stem Cell-derived Mesoangioblast-like Myogenic Progenitors in Mouse Models of Muscle Regeneration10:03

Transplantation of Induced Pluripotent Stem Cell-derived Mesoangioblast-like Myogenic Progenitors in Mouse Models of Muscle Regeneration

Published on: January 20, 2014

10.1K
FACS-Isolation and Culture of Fibro-Adipogenic Progenitors and Muscle Stem Cells from Unperturbed and Injured Mouse Skeletal Muscle11:25

FACS-Isolation and Culture of Fibro-Adipogenic Progenitors and Muscle Stem Cells from Unperturbed and Injured Mouse Skeletal Muscle

Published on: June 8, 2022

7.2K

Related Concept Videos

Classification of Skeletal Muscle Fibers01:48

Classification of Skeletal Muscle Fibers

Skeletal muscles continuously produce ATP to provide the energy that enables muscle contractions. Skeletal muscle fibers can be categorized into three types based on differences in their contraction speed and how they produce ATP, as well as physical differences related to these factors. Most human muscles contain all three muscle fiber types, albeit in varying proportions.
Slow-Twitch Muscle Fibers
Slow oxidative, muscle fibers appear red due to large numbers of capillaries and high levels of...
59.6K
Stem Cell Therapy for Tissue Regeneration01:21

Stem Cell Therapy for Tissue Regeneration

Stem cell therapy is a method used in regenerative medicine to repair and restore function to damaged tissues and organs. Stem cells have the potential to proliferate and differentiate into various tissue types, making them ideal candidates for tissue regeneration. For example, hematopoietic stem cell transplants are commonly used in blood cancer treatment to replenish damaged bone marrow and restore healthy blood cells.
Types of Stem Cells used in Stem Cell Therapy
The two main cell...
4.7K
Muscles of the Eye01:20

Muscles of the Eye

The muscles of the eye are sophisticated structures that control eye movement and focus, allowing for the precise and rapid adjustments necessary for vision. The human eye is controlled by ten muscles — six extraocular muscles, three intraocular muscles, and one primary eyelid retractor muscle.
Extraocular Muscles
The six extraocular muscles surround the eyeball and control its movements. They are responsible for a wide range of eye motions, including looking up, down, left, right, and...
4.6K
Muscles that Move the Head01:19

Muscles that Move the Head

The muscles that move the head are a dynamic and complex group of structures that work together to facilitate a wide range of head movements, including rotation, flexion, extension, and lateral bending.
The bilateral sternocleidomastoid, or SCM, and the suprahyoid and infrahyoid muscles are significant head flexors. The SCM muscles originate at the sternum and clavicle and attach to the mastoid process of the temporal bone. The SCM contracts bilaterally to bend the head forward, whereas...
6.1K
Muscles of the Abdomen01:21

Muscles of the Abdomen

The abdominal wall encircles the abdominal cavity, providing flexible protection and shielding the internal organs from harm. It is bordered at the top by the xiphoid process and costal margins, at the back by the vertebral column, and at the bottom by the pelvic bones and inguinal ligament. The abdominal wall is divided into two regions — the anterolateral and posterior regions.
Anterolateral Region
The anterolateral region comprises five paired muscles classified into the lateral and...
3.6K
Muscles that Move the Arm01:31

Muscles that Move the Arm

Nine muscles are involved in arm movements. Two of these, the pectoralis major and latissimus dorsi, originate from the axial skeleton and are called axial muscles. The other seven originate from the scapula and are called the scapular muscles.
The pectoralis major has two origins. Its clavicular head originates on the medial half of the clavicle. In contrast, the sternocostal head originates on the costal cartilages of ribs 1-6, the sternum, and the aponeurosis of the external oblique of the...
4.9K