Abstract:
OBJECTIVE:To assess the changes of muscle-related biomarkers at the early stage of amyotrophic lateral sclerosis, and to confirm these findings in an experimental animal model. METHODS:Thirty-nine subjects with sporadic amyotrophic lateral sclerosis and 20 healthy controls were enrolled and longitudinally evaluated. We evaluated serum creatine kinase and creatinine levels and appendicular lean soft-tissue mass using dual X-ray absorptiometry. The levels of biomarkers at early ALS stages were estimated using linear mixed models with unstructured correlation and random intercepts. We also analyzed the longitudinal changes of serum creatine kinase and creatinine, together with the mRNA levels of acetylcholine receptor subunit γ (Chrng) and muscle-associated receptor tyrosine kinase, markers of denervation, in the gastrocnemius muscle of superoxide dismutase 1 (SOD1)G93A transgenic mice, an animal model of amyotrophic lateral sclerosis. RESULTS:The estimated levels of creatine kinase were higher in subjects with amyotrophic lateral sclerosis at the early stage than in healthy controls, although the estimated appendicular lean soft-tissue mass and creatinine levels were equivalent between both groups, suggesting that the elevation of creatine kinase precedes both muscular atrophy and subjective motor symptoms in sporadic amyotrophic lateral sclerosis. In SOD1G93A mice, the serum levels of creatine kinase were elevated at 9 weeks of age (peri-onset) when Chrng started to be up-regulated, and were then down-regulated at 15 weeks of age, consistent with the clinical data from patients with sporadic amyotrophic lateral sclerosis. INTERPRETATION:Creatine kinase elevation precedes muscular atrophy and reflects muscle denervation at the early stage.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ito D,Hashizume A,Hijikata Y,Yamada S,Iguchi Y,Iida M,Kishimoto Y,Moriyoshi H,Hirakawa A,Katsuno Mdoi
10.1007/s00415-019-09507-6subject
Has Abstractpub_date
2019-12-01 00:00:00pages
2952-2961issue
12eissn
0340-5354issn
1432-1459pii
10.1007/s00415-019-09507-6journal_volume
266pub_type
杂志文章abstract::The greatest unmet need in multiple sclerosis (MS) are treatments that delay, prevent or reverse progression. One of the most tractable strategies to achieve this is to therapeutically enhance endogenous remyelination; doing so restores nerve conduction and prevents neurodegeneration. The biology of remyelination-cent...
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pub_type: 杂志文章,评审
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