Ultrastructural changes in myocardial cells of mice with dysferlinopathy (Bla/J line)
- Authors: Limaev I.S.1, Yakovlev I.A.2, Chekmareva I.A.1,3, Bardakov S.N.4, Emelin A.M.1, Savelyeva M.A.5, Deykin A.V.6, Deev R.V.1,2
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Affiliations:
- Petrovsky National Research Centre of Surgery
- Genotarget LLC
- A.V. Vishnevsky National Medical Research Center of Surgery
- Military Medical Academy named after S.M. Kirov
- North-Western State Medical University named after I.I. Mechnikov
- Belgorod National Research University
- Issue: Vol 162, No 4 (2024)
- Pages: 390-400
- Section: Original Study Articles
- Submitted: 06.09.2024
- Accepted: 28.12.2024
- Published: 28.12.2024
- URL: https://j-morphology.com/1026-3543/article/view/635735
- DOI: https://doi.org/10.17816/morph.635735
- ID: 635735
Cite item
Abstract
BACKGROUND: Striated cardiac muscle tissue in dysferlinopathy, a rare hereditary muscular dystrophy, has been the subject of limited research. Dysferlinopathy is traditionally considered a disease that predominantly affects skeletal muscles, as clinically significant heart failure is rare in affected individuals. However, myocardial involvement due to hereditary dysferlin deficiency has been described in only a few studies. The development of heart failure in these patients may result from both circulatory remodeling due to hypodynamia and direct myocardial damage. Structural changes observed in Bla/J mice with dysferlinopathy provide evidence for direct myocardial damage. However, submicroscopic alterations in cardiomyocytes and stromal myocardial cells (fibroblasts, endothelial cells, telocytes), as well as their role in the pathomorphogenesis of dysferlinopathy, remain insufficiently studied.
AIM: To characterize the ultrastructure of cardiomyocytes and stromal myocardial cells in the left ventricle of dysferlin-deficient Bla/J mice.
METHODS: Myocardial left ventricle fragments from Bla/J and C57BL/6 (control group) mice at 3, 6, 9, and 12 months of age were fixed and embedded in Araldite resin. Ultrathin sections (50–100 nm) were prepared, stained using Reynolds’ method, and examined via transmission electron microscopy.
RESULTS: Ultrastructural changes in the myocardium of Bla/J dysferlin-deficient mice included: destruction of the sarcolemma and intercalated discs; expansion and vacuolization of the sarcoplasmic reticulum; mitochondrial polymorphism. Additionally, myelin-like structures were detected in subsarcolemmal spaces and sarcoplasmic reticulum cisterns. In dysferlin-deficient mice, telocytes exhibited signs of degeneration. In contrast, the control group (C57BL/6 mice) showed no significant ultrastructural changes.
CONCLUSION: Ultrastructural evidence of myocardial damage in dysferlin-deficient Bla/J mice suggests a potential role of dysferlin in maintaining the structural integrity of cardiomyocytes and stromal cells.
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About the authors
Igor S. Limaev
Petrovsky National Research Centre of Surgery
Author for correspondence.
Email: is.limaev@proton.me
ORCID iD: 0000-0002-0994-9787
SPIN-code: 4909-6550
Russian Federation, Moscow
Ivan A. Yakovlev
Genotarget LLC
Email: ivan@ivan-ya.ru
ORCID iD: 0000-0001-8127-4078
SPIN-code: 8222-2234
MD, Cand. Sci. (Medicine)
Russian Federation, MoscowIrina A. Chekmareva
Petrovsky National Research Centre of Surgery; A.V. Vishnevsky National Medical Research Center of Surgery
Email: chia236@mail.ru
ORCID iD: 0000-0003-0126-4473
SPIN-code: 5994-7650
Dr. Sci. (Biology)
Russian Federation, Moscow; MoscowSergey N. Bardakov
Military Medical Academy named after S.M. Kirov
Email: epistaxis@mail.ru
ORCID iD: 0000-0002-3804-6245
SPIN-code: 2351-4096
MD, Cand. Sci. (Medicine)
Russian Federation, Saint PetersburgAleksey M. Emelin
Petrovsky National Research Centre of Surgery
Email: eamar40rn@gmail.com
ORCID iD: 0000-0003-4109-0105
SPIN-code: 5605-1140
Russian Federation, Moscow
Maria A. Savelyeva
North-Western State Medical University named after I.I. Mechnikov
Email: savelyeva.mariaanat@yandex.ru
ORCID iD: 0009-0008-5667-115X
SPIN-code: 9935-5416
Russian Federation, Saint Petersburg
Alexey V. Deykin
Belgorod National Research University
Email: alexei@deikin.ru
ORCID iD: 0000-0001-9960-0863
SPIN-code: 8371-5197
Cand. Sci. (Biology)
Russian Federation, BelgorodRoman V. Deev
Petrovsky National Research Centre of Surgery; Genotarget LLC
Email: romdey@gmail.com
ORCID iD: 0000-0001-8389-3841
SPIN-code: 2957-1687
Cand. Sci. (Medicine)
Russian Federation, Moscow; MoscowReferences
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