Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II
Abstract Electrical impedance methods, including electrical impedance myography, are increasingly being used as biomarkers of muscle health since they measure passive electrical properties of muscle that alter in disease. One disorder, Pompe Disease (Glycogen storage disease type II (GSDII)), remain...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Nature Portfolio
2022-03-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-022-09328-0 |
| _version_ | 1811272067307274240 |
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| author | Janice A. Nagy Carson Semple Daniela Riveros Benjamin Sanchez Seward B. Rutkove |
| author_facet | Janice A. Nagy Carson Semple Daniela Riveros Benjamin Sanchez Seward B. Rutkove |
| author_sort | Janice A. Nagy |
| collection | DOAJ |
| description | Abstract Electrical impedance methods, including electrical impedance myography, are increasingly being used as biomarkers of muscle health since they measure passive electrical properties of muscle that alter in disease. One disorder, Pompe Disease (Glycogen storage disease type II (GSDII)), remains relatively unstudied. This disease is marked by dramatic accumulation of intracellular myofiber glycogen. Here we assessed the electrical properties of skeletal muscle in a model of GSDII, the Pompe 6neo/6neo (Pompe) mouse. Ex vivo impedance measurements of gastrocnemius (GA) were obtained using a dielectric measuring cell in 30-week-old female Pompe (N = 10) and WT (N = 10) mice. Longitudinal and transverse conductivity, σ, and the relative permittivity, εr, and Cole–Cole complex resistivity parameters at 0 Hz and infinite frequency, ρo and ρ∞, respectively, and the intracellular resistivity, ρintracellular were determined from the impedance data. Glycogen content (GC) was visualized histologically and quantified biochemically. At frequencies > 1 MHz, Pompe mice demonstrated significantly decreased longitudinal and transverse conductivity, increased Cole–Cole parameters, ρo and ρo-ρ∞, and decreased ρintracellular. Changes in longitudinal conductivity and ρintracellular correlated with increased GC in Pompe animals. Ex vivo high frequency impedance measures are sensitive to alterations in intracellular myofiber features considered characteristic of GSDII, making them potentially useful measures of disease status. |
| first_indexed | 2024-04-12T22:33:43Z |
| format | Article |
| id | doaj.art-357b7276b11e41ce925ada2ef913cb2d |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-04-12T22:33:43Z |
| publishDate | 2022-03-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-357b7276b11e41ce925ada2ef913cb2d2022-12-22T03:13:55ZengNature PortfolioScientific Reports2045-23222022-03-0112111110.1038/s41598-022-09328-0Altered electrical properties in skeletal muscle of mice with glycogen storage disease type IIJanice A. Nagy0Carson Semple1Daniela Riveros2Benjamin Sanchez3Seward B. Rutkove4Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical SchoolDepartment of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical SchoolDepartment of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical SchoolDepartment of Electrical and Computer Engineering, University of UtahDepartment of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical SchoolAbstract Electrical impedance methods, including electrical impedance myography, are increasingly being used as biomarkers of muscle health since they measure passive electrical properties of muscle that alter in disease. One disorder, Pompe Disease (Glycogen storage disease type II (GSDII)), remains relatively unstudied. This disease is marked by dramatic accumulation of intracellular myofiber glycogen. Here we assessed the electrical properties of skeletal muscle in a model of GSDII, the Pompe 6neo/6neo (Pompe) mouse. Ex vivo impedance measurements of gastrocnemius (GA) were obtained using a dielectric measuring cell in 30-week-old female Pompe (N = 10) and WT (N = 10) mice. Longitudinal and transverse conductivity, σ, and the relative permittivity, εr, and Cole–Cole complex resistivity parameters at 0 Hz and infinite frequency, ρo and ρ∞, respectively, and the intracellular resistivity, ρintracellular were determined from the impedance data. Glycogen content (GC) was visualized histologically and quantified biochemically. At frequencies > 1 MHz, Pompe mice demonstrated significantly decreased longitudinal and transverse conductivity, increased Cole–Cole parameters, ρo and ρo-ρ∞, and decreased ρintracellular. Changes in longitudinal conductivity and ρintracellular correlated with increased GC in Pompe animals. Ex vivo high frequency impedance measures are sensitive to alterations in intracellular myofiber features considered characteristic of GSDII, making them potentially useful measures of disease status.https://doi.org/10.1038/s41598-022-09328-0 |
| spellingShingle | Janice A. Nagy Carson Semple Daniela Riveros Benjamin Sanchez Seward B. Rutkove Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II Scientific Reports |
| title | Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II |
| title_full | Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II |
| title_fullStr | Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II |
| title_full_unstemmed | Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II |
| title_short | Altered electrical properties in skeletal muscle of mice with glycogen storage disease type II |
| title_sort | altered electrical properties in skeletal muscle of mice with glycogen storage disease type ii |
| url | https://doi.org/10.1038/s41598-022-09328-0 |
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