ArticleNan fang yi ke da xue xue bao = Journal of Southern Medical University2023
[Molecular pathological mechanism of liver metabolic disorder in mice with severe spinal muscular atrophy].
Article in Nan fang yi ke da xue xue bao = Journal of Southern Medical University, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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5 authors at 1 institution in 1 country.
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Abstract
objectiveTo explore the molecular pathological mechanism of liver metabolic disorder in severe spinal muscular atrophy (SMA).
methodsThe transgenic mice with type Ⅰ SMA (
resultsThe neonatal mice with type Ⅰ SMA showed normal milk suckling behavior but had lower body weight than the littermate control mice on the second day after birth. Intraperitoneal injection of glucose solution every 12 h significantly improved the median survival time of type Ⅰ SMA mice from 9±1.3 to 11± 1.5 days (
conclusionType Ⅰ SMA mice have liver metabolic disorder, and the down-regulation of the target genes of PPARα related to lipid and glucose metabolism due to persistent DNA methylation contributes to the progression of SMA.
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