Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat
Abstract Background Duchenne muscular dystrophy (DMD) is an X-linked muscle disease caused by a complete lack of dystrophin, which stabilizes the plasma membrane of myofibers. The orofacial function is affected in an advanced stage of DMD and this often leads to an eating disorder such as dysphagia....
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2022-10-01
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| Series: | Skeletal Muscle |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13395-022-00307-7 |
| _version_ | 1797986036310081536 |
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| author | Keitaro Yamanouchi Yukie Tanaka Masanari Ikeda Shizuka Kato Ryosuke Okino Hiroki Nishi Fumihiko Hakuno Shin-Ichiro Takahashi James Chambers Takashi Matsuwaki Kazuyuki Uchida |
| author_facet | Keitaro Yamanouchi Yukie Tanaka Masanari Ikeda Shizuka Kato Ryosuke Okino Hiroki Nishi Fumihiko Hakuno Shin-Ichiro Takahashi James Chambers Takashi Matsuwaki Kazuyuki Uchida |
| author_sort | Keitaro Yamanouchi |
| collection | DOAJ |
| description | Abstract Background Duchenne muscular dystrophy (DMD) is an X-linked muscle disease caused by a complete lack of dystrophin, which stabilizes the plasma membrane of myofibers. The orofacial function is affected in an advanced stage of DMD and this often leads to an eating disorder such as dysphagia. Dysphagia is caused by multiple etiologies including decreased mastication and swallowing. Therefore, preventing the functional declines of mastication and swallowing in DMD is important to improve the patient’s quality of life. In the present study, using a rat model of DMD we generated previously, we performed analyses on the masseter and tongue muscles, both are required for proper eating function. Methods Age-related changes of the masseter and tongue muscle of DMD rats were analyzed morphometrically, histologically, and immunohistochemically. Also, transcription of cellular senescent markers, and utrophin (Utrn), a functional analog of dystrophin, was examined. Results The masseter muscle of DMD rats showed progressive dystrophic changes as observed in their hindlimb muscle, accompanied by increased transcription of p16 and p19. On the other hand, the tongue of DMD rats showed macroglossia due to hypertrophy of myofibers with less dystrophic changes. Proliferative activity was preserved in the satellite cells from the tongue muscle but was perturbed severely in those from the masseter muscle. While Utrn transcription was increased in the masseter muscle of DMD rats compared to WT rats, probably due to a compensatory mechanism, its level in the tongue muscle was comparable between WT and DMD rats and was similar to that in the masseter muscle of DMD rats. Conclusions Muscular dystrophy is less advanced in the tongue muscle compared to the masseter muscle in the DMD rat. |
| first_indexed | 2024-04-11T07:26:46Z |
| format | Article |
| id | doaj.art-aeae3a048a30439680dbbe41dd4509b4 |
| institution | Directory Open Access Journal |
| issn | 2044-5040 |
| language | English |
| last_indexed | 2024-04-11T07:26:46Z |
| publishDate | 2022-10-01 |
| publisher | BMC |
| record_format | Article |
| series | Skeletal Muscle |
| spelling | doaj.art-aeae3a048a30439680dbbe41dd4509b42022-12-22T04:37:02ZengBMCSkeletal Muscle2044-50402022-10-0112111310.1186/s13395-022-00307-7Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy ratKeitaro Yamanouchi0Yukie Tanaka1Masanari Ikeda2Shizuka Kato3Ryosuke Okino4Hiroki Nishi5Fumihiko Hakuno6Shin-Ichiro Takahashi7James Chambers8Takashi Matsuwaki9Kazuyuki Uchida10Laboratory of Veterinary Physiology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Physiology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Physiology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Pathology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Animal Cell Regulation, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Animal Cell Regulation, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Animal Cell Regulation, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Animal Cell Regulation, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Pathology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Physiology, Graduate School of Agricultural and Life Sciences, The University of TokyoLaboratory of Veterinary Pathology, Graduate School of Agricultural and Life Sciences, The University of TokyoAbstract Background Duchenne muscular dystrophy (DMD) is an X-linked muscle disease caused by a complete lack of dystrophin, which stabilizes the plasma membrane of myofibers. The orofacial function is affected in an advanced stage of DMD and this often leads to an eating disorder such as dysphagia. Dysphagia is caused by multiple etiologies including decreased mastication and swallowing. Therefore, preventing the functional declines of mastication and swallowing in DMD is important to improve the patient’s quality of life. In the present study, using a rat model of DMD we generated previously, we performed analyses on the masseter and tongue muscles, both are required for proper eating function. Methods Age-related changes of the masseter and tongue muscle of DMD rats were analyzed morphometrically, histologically, and immunohistochemically. Also, transcription of cellular senescent markers, and utrophin (Utrn), a functional analog of dystrophin, was examined. Results The masseter muscle of DMD rats showed progressive dystrophic changes as observed in their hindlimb muscle, accompanied by increased transcription of p16 and p19. On the other hand, the tongue of DMD rats showed macroglossia due to hypertrophy of myofibers with less dystrophic changes. Proliferative activity was preserved in the satellite cells from the tongue muscle but was perturbed severely in those from the masseter muscle. While Utrn transcription was increased in the masseter muscle of DMD rats compared to WT rats, probably due to a compensatory mechanism, its level in the tongue muscle was comparable between WT and DMD rats and was similar to that in the masseter muscle of DMD rats. Conclusions Muscular dystrophy is less advanced in the tongue muscle compared to the masseter muscle in the DMD rat.https://doi.org/10.1186/s13395-022-00307-7Duchenne muscular dystrophyMasseter muscleTongueMacroglossiaRat |
| spellingShingle | Keitaro Yamanouchi Yukie Tanaka Masanari Ikeda Shizuka Kato Ryosuke Okino Hiroki Nishi Fumihiko Hakuno Shin-Ichiro Takahashi James Chambers Takashi Matsuwaki Kazuyuki Uchida Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat Skeletal Muscle Duchenne muscular dystrophy Masseter muscle Tongue Macroglossia Rat |
| title | Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat |
| title_full | Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat |
| title_fullStr | Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat |
| title_full_unstemmed | Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat |
| title_short | Macroglossia and less advanced dystrophic change in the tongue muscle of the Duchenne muscular dystrophy rat |
| title_sort | macroglossia and less advanced dystrophic change in the tongue muscle of the duchenne muscular dystrophy rat |
| topic | Duchenne muscular dystrophy Masseter muscle Tongue Macroglossia Rat |
| url | https://doi.org/10.1186/s13395-022-00307-7 |
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