Pulsatile Myofilament Activity in Myotrem Myopathy Associated with Myogenic Tremor
Abstract
1. Introduction
2. Results
2.1. Contractile Properties of Soleus Muscle Fibers
2.2. Stretch Activation Properties of Soleus Muscle Fibers
2.3. Pulsatile Activity of a Subpopulation of E248K KI Soleus Muscle Fibers
2.4. Fiber-Type Specific Stretch Activation Properties
3. Discussion
4. Materials and Methods
4.1. Study Approval
4.2. Solutions
4.3. Fiber Preparation and Experimental Setup
4.4. Slack–Re-Stretch Protocol
4.5. Step–Stretch Protocol and Data Analysis
4.6. Single Fiber Immunoblot Analysis
4.7. Statistical Analysis
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
CNS | Central nervous system |
EDL | Extensor digitorum longus |
kdf | Delayed force development rate |
KI | Knock-In |
krel | Rapid force decay rate |
MHC | Myosin Heavy Chain |
MyBP-C | Myosin binding protein-C |
PO | Pre-stretch steady state tension |
PSS | Stretch-induced steady state tension |
PTO | Stretch activation |
P1 | Maximum force peak |
P2 | Minimum force decay |
sMyBP-C | Slow skeletal myosin binding protein-C |
SPOC | Spontaneous oscillatory contraction |
WT | Wild type |
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Fiber Length (µm) | Fiber Widthair (µm) | Sarcomere Length (µm) | Passive Tension (kN/m2) | Maximal Tension (kN/m2) | pCa 4.5 ktr (s−1) | ||
---|---|---|---|---|---|---|---|
WT | n′ = 8 | 911 ±71 | 39 ± 2 | 2.36 ± 0.03 | 3.29 ± 0.49 | 93 ± 5 | 10.5 ± 1.1 |
KI | n′ = 11 | 939 ± 60 | 44 ± 1 | 2.32 ± 0.03 | 2.56 ± 0.32 | 64 ± 6 * | 10.3 ± 1.1 |
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Mariano, J.M.; Hanft, L.M.; Cho, S.; Ward, C.W.; McDonald, K.S.; Kontrogianni-Konstantopoulos, A. Pulsatile Myofilament Activity in Myotrem Myopathy Associated with Myogenic Tremor. Int. J. Mol. Sci. 2025, 26, 5252. https://doi.org/10.3390/ijms26115252
Mariano JM, Hanft LM, Cho S, Ward CW, McDonald KS, Kontrogianni-Konstantopoulos A. Pulsatile Myofilament Activity in Myotrem Myopathy Associated with Myogenic Tremor. International Journal of Molecular Sciences. 2025; 26(11):5252. https://doi.org/10.3390/ijms26115252
Chicago/Turabian StyleMariano, Jennifer Megan, Laurin M. Hanft, Suhan Cho, Christopher W. Ward, Kerry S. McDonald, and Aikaterini Kontrogianni-Konstantopoulos. 2025. "Pulsatile Myofilament Activity in Myotrem Myopathy Associated with Myogenic Tremor" International Journal of Molecular Sciences 26, no. 11: 5252. https://doi.org/10.3390/ijms26115252
APA StyleMariano, J. M., Hanft, L. M., Cho, S., Ward, C. W., McDonald, K. S., & Kontrogianni-Konstantopoulos, A. (2025). Pulsatile Myofilament Activity in Myotrem Myopathy Associated with Myogenic Tremor. International Journal of Molecular Sciences, 26(11), 5252. https://doi.org/10.3390/ijms26115252