Ultrastructural Characterisation of Quadriceps Muscle In Adult Ts1Cje Mouse Model of Down Syndrome
| Autor: | Bala, Usman, S S Bello, F Othman, M Lai, K, Ling, Cheah P |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| DOI: | 10.5281/zenodo.5578959 |
| Popis: | Background Down syndrome (DS) is a chromosomal anomaly due to triplication of human chromosome 21 (Hsa21), a condition also known as Trisomy 21. It is characterized by several phenotypes such as motor dysfunction, intellectual disability and systems anomalies. Ts1Cje, a mouse model of DS carries a shorter region of Mmu16 with approximately 85 genes homologous to genes located on Hsa21 and is well reported to exhibit various phenotypes seen in DS. Objectives: This study investigated the ultrastructural morphology of quadriceps muscle in Ts1Cje mice. Methods: PCR genotyping was performed to determine the genotype of the moue. Quadriceps muscles were excised and processed for transmission electron microscope images. Morphometric evaluation of the fibre cross-sectional area, nuclear area, sarcomere length and mitochondrial population was performed. Results: The result showed no morphological difference in the general ultrastructural organisation of quadriceps between both genotypes. The myofibre maintained its characteristic morphological features and the cytoplasm is filled with myofibrils and mitochondria. Morphometric analysis indicated that the subsarcolemmal mitochondrial population was significantly reduced (P=0.0045) in the Ts1Cje mice as compared with the wild type mice. Also, the number of intermyofibrillar mitochondria differs significantly (P=0.0001) between the two genotypes. Conclusion: It is therefore concluded that the reduced number of mitochondria in Ts1Cje mice would further contribute to the functional defects associated with mitochondria seen in DS. {"references":["Egan JFX, Benn PA, Zelop CM, Bolnick A, Gianferrari E, Borgida AF. Down syndrome births in the United States from 1989 to 2001. Am J Obstet Gynecol. 2004;191(3):1044-1048"]} |
| Databáze: | OpenAIRE |
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