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Muscle death participates in myofibrillar abnormalities in FHL1 knockout mice.
- Source :
-
Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2020 Feb 26; Vol. 523 (1), pp. 105-111. Date of Electronic Publication: 2019 Dec 10. - Publication Year :
- 2020
-
Abstract
- Background: Mutations in the four and-a-half LIM domain protein 1 (FHL1) gene or FHL1 protein deletion have been identified as the cause of rare hereditary myopathies or cardiomyopathies. In our previous study, autophagy activation was associated with myofibrillar abnormalities in FHL1 knockout (KO) mice. P2RX7 induces cell death, such as autophagy, pyroptosis or apoptosis via cell-specific downstream signaling; however, the roles of P2RX7 in pyroptosis or apoptosis in myofibrillar abnormalities in FHL1 KO mice have not been well elucidated.<br />Methods: In this study, skeletal muscle and heart of 2.5 months old WT and FHL1 KO male mice histomorphology were examined by hematoxylin and eosin staining. The indicators for pyroptosis (NLRP3; ASC; cleaved-caspase1; IL-1β), apoptosis (Apaf-1; Bcl-2; caspase9; cleaved-caspase3), and P2RX7 were detected in the triceps (Tri), tibialis anterior muscles (TA), and heart by western blot and/or immunohistochemistry in WT and FHL1 KO male mice.<br />Results: Indicators for pyroptosis (ASC; cleaved-caspase1; IL-1β) and apoptosis (Apaf-1 and cleaved-caspase3), as well as P2RX7 were upregulated in Tri, tibialis TA, and heart in FHL1 KO mice, indicating pyroptosis and apoptosis play important roles in myofibrillar abnormalities in FHL1 KO mice.<br />Conclusions: P2RX7 may participate in myofibrillar abnormalities by activating pyroptosis and apoptosis in FHL1 KO mice. These findings have basic implications for the understanding of myopathies induced by FHL1 deficiency and provide new avenues for the treatment of these hereditary myopathies by modulating P2RX7.<br />Competing Interests: Declaration of competing interest The authors declare no conflict of interest.<br /> (Copyright © 2019 Elsevier Inc. All rights reserved.)
- Subjects :
- Animals
Male
Mice
Mice, Knockout
Muscle, Skeletal metabolism
Muscular Diseases pathology
Receptors, Purinergic P2X7 metabolism
Apoptosis
Intracellular Signaling Peptides and Proteins deficiency
Intracellular Signaling Peptides and Proteins metabolism
LIM Domain Proteins deficiency
LIM Domain Proteins metabolism
Muscle Proteins deficiency
Muscle Proteins metabolism
Muscular Diseases metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1090-2104
- Volume :
- 523
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Biochemical and biophysical research communications
- Publication Type :
- Academic Journal
- Accession number :
- 31836140
- Full Text :
- https://doi.org/10.1016/j.bbrc.2019.12.026