Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model

Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16...

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Veröffentlicht in:	PloS one 2018-05, Vol.13 (5), p.e0197711-e0197711
Hauptverfasser:	Bala, Usman, Leong, Melody Pui-Yee, Lim, Chai Ling, Shahar, Hayati Kadir, Othman, Fauziah, Lai, Mei-I, Law, Zhe-Kang, Ramli, Khairunnisa, Htwe, Ohnmar, Ling, King-Hwa, Cheah, Pike-See
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biology and Life Sciences Care and treatment Chromosome 21 Chromosomes Complications and side effects Defects Development and progression Disease Models, Animal Down syndrome Down Syndrome - complications Down Syndrome - metabolism Down Syndrome - pathology Down's syndrome Electron Transport Complex IV - metabolism Etiology Female Fetuses Fibers Gene Expression Regulation Genetic disorders Genetics Genomics Genotype Grip strength Hand Strength - physiology Health sciences Homology Hypotonia Latency Male Medicine Medicine and Health Sciences Mice Motor ability Motor Activity - physiology Motor task performance Muscle Fibers, Skeletal - metabolism Muscle Fibers, Skeletal - pathology Muscle strength Muscle Weakness - complications Muscle Weakness - metabolism Muscle Weakness - pathology Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscles Muscular strength Musculoskeletal diseases Musculoskeletal system MyoD protein MyoD Protein - metabolism Myogenic Regulatory Factor 5 - metabolism Nerve conduction Neural Conduction - physiology Phenotypes Quadriceps muscle Research and Analysis Methods Rodents Skeletal muscle Trisomy Velocity
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Beschreibung
Zusammenfassung:	Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16) homologous to Hsa21, is well reported to exhibit various typical neuropathological features seen in individuals with DS. This study investigated the role of skeletal muscles and peripheral nerve defects in contributing to muscle weakness in Ts1Cje mice. Assessment of the motor performance showed that, the forelimb grip strength was significantly (P
ISSN:	1932-6203 1932-6203
DOI:	10.1371/journal.pone.0197711