Related Experiment Video

Updated: Jul 25, 2026

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
14:10

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Published on: January 31, 2013

An insulin receptor defect in murine muscular dystrophy

W W Hofmann

    Muscle & Nerve
    |October 1, 1984
    PubMed

    Insights

    Insulin receptor affinity is reduced in dystrophic mouse muscles, impairing nutrient uptake. This suggests a genetic defect in receptor proteins may cause muscular dystrophy abnormalities.

    Area of Science:

    • Biochemistry
    • Molecular Biology
    • Physiology

    Background:

    • Muscular dystrophy in mice (129 ReJ strain) exhibits biochemical and electrophysiological abnormalities.
    • Insulin plays a crucial role in cellular nutrient uptake and metabolic regulation.

    Purpose of the Study:

    • To investigate insulin binding and postbinding effects on dystrophic mouse soleus muscles.
    • To compare these effects with those in healthy control mice.

    Main Methods:

    • Utilized Iodine-125 (I125) labeled insulin for binding studies.
    • Assessed postbinding effects on nutrient uptake, including 2-deoxyglucose (2-DG) and aminoisobutyric acid (AIB).
    • Compared results from dystrophic mice with sex- and weight-matched controls.

    Main Results:

    • Insulin receptor affinity was found to be lower in dystrophic muscles compared to controls at physiological hormone concentrations.
    • Insulin-dependent uptake of 2-deoxyglucose (2-DG) and aminoisobutyric acid (AIB) was impaired in dystrophic muscles.
    • Data indicate reduced insulin signaling efficacy in dystrophic muscle tissue.

    Conclusions:

    • Genetic defects in insulin receptor proteins may underlie the observed abnormalities in murine muscular dystrophy.
    • Impaired insulin receptor function could contribute to the biochemical and electrophysiological deficits seen in dystrophy.
    • Targeting insulin receptor pathways may offer therapeutic potential for muscular dystrophy.

    More Related Videos

    Assessing Functional Performance in the Mdx Mouse Model10:32

    Assessing Functional Performance in the Mdx Mouse Model

    Published on: March 27, 2014

    Single Myofiber Isolation and Culture from a Murine Model of Emery-Dreifuss Muscular Dystrophy in Early Post-Natal Development08:07

    Single Myofiber Isolation and Culture from a Murine Model of Emery-Dreifuss Muscular Dystrophy in Early Post-Natal Development

    Published on: July 1, 2020

    Related Concept Videos

    Satellite Stem Cells and Muscular Dystrophy01:21

    Satellite Stem Cells and Muscular Dystrophy

    Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
    Insulin: The Receptor and Signaling Pathways01:28

    Insulin: The Receptor and Signaling Pathways

    Insulin action is mediated through a receptor tyrosine kinase, akin to the IGF-1 receptor. The number of receptors per cell varies significantly, from 40 on erythrocytes to 300,000 on adipocytes and hepatocytes. The insulin receptor consists of linked α/β subunit dimers, forming a heterotetramer glycoprotein with two extracellular α subunits and two β subunits spanning the membrane. The α subunits inhibit the inherent tyrosine kinase activity of the β subunits, but this inhibition is released...